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A 44-year-old woman with type 1 diabetes mellitus has not
attended the diabetic clinic for five years.
Test Analysis
Examination shows no abnormalities.

Investigations show:

Haemoglobin 90 g/L (115 - 165)

MCV 94 fL (80 - 96)

Haematocrit 28% -

87 mmol/mol (20-42)
HbA1c
10.1% (3.8 - 6.4)

A blood smear shows normochromic, normocytic anaemia.

Which of the following is the most likely cause?

(Please select 1 option)



Acute blood loss Score:


Chronic lymphocytic leukaemia (CLL) Total Answered:


Erythropoietin deficiency
Question Navigator

Microangiopathic haemolysis


Sideroblastic anaemia
Tags

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A 44-year-old woman with type 1 diabetes mellitus has not
attended the diabetic clinic for five years.
Answer Statistics
Examination shows no abnormalities.

Investigations show: Test Analysis

Haemoglobin 90 g/L (115 - 165)

MCV 94 fL (80 - 96)

Haematocrit 28% -

87 mmol/mol (20-42)
HbA1c
10.1% (3.8 - 6.4)

A blood smear shows normochromic, normocytic anaemia.

Which of the following is the most likely cause?

(Please select 1 option)


Acute blood loss
Incorrect answer selected


Chronic lymphocytic leukaemia (CLL)
Score:
Erythropoietin deficiency
This is the correct answer
Total Answered:

Microangiopathic haemolysis


Sideroblastic anaemia
Feedback

The most likely cause is progressive renal failure which leads


to reduced release of erythropoietin from the Question Navigator
kidneys.
Revision Notes
Sideroblastic anaemia (myelodysplasia) is seen in older age
groups.

CLL or microangiopathic haemolysis are possible causes but


unlikely. Tags

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A 60-year-old Chinese man has been started by his general
practitioner on quinine for leg cramps.
Test Analysis
He presents, a week later, with five days of darkened urine and
two days of increasing breathlessness, back pain
and fatigue.

Investigations show a haemoglobin of 70 g/L (130-180) and raised


reticulocyte count.

Which of the following best explain this drug reaction?

(Please select 1 option)



Autoimmune haemolytic anaemia


Glucose-6-phosphate dehydrogenase deficiency


Hereditary spherocytosis


Pyruvate kinase deficiency


Sickle cell disease

Score:

Total Answered:
Submit answer
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A 60-year-old Chinese man has been started by his general
practitioner on quinine for leg cramps.
Answer Statistics
He presents, a week later, with five days of darkened urine and
two days of increasing breathlessness, back pain
and fatigue. Test Analysis
Investigations show a haemoglobin of 70 g/L (130-180) and raised
reticulocyte count.

Which of the following best explain this drug reaction?

(Please select 1 option)


Autoimmune haemolytic anaemia
Incorrect answer selected

Glucose-6-phosphate dehydrogenase deficiency


This is the correct answer


Hereditary spherocytosis


Pyruvate kinase deficiency


Sickle cell disease

G6PDH (X linked recessive) is seen in African, Mediterranean,


Iraqi, Jew, South East Asian and Chinese people
and predisposes to
a haemolytic anaemia reaction with drugs or infection. Score:

Implicated drugs include: Total Answered:


Aspirin
Sulphonamides
Feedback
Antimalarials, and
Quinine/quinidine.
Question Navigator
The haemolytic anaemia is non-immune (direct antiglobulin test
[DAT] negative).

Pyruvate kinase deficiency is autosomal recessive and presents


as a chronic haemolytic anaemia exacerbated Revision Notes
by viral
infections.

Hereditary spherocytosis is characterised by variable chronic


non-immune haemolysis exacerbated by infections. Tags

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A 78-year-old lady is admitted to hospital for a right
hemicolectomy for carcinoma of the caecum.
Test Analysis
She has a history of osteoarthritis for which she takes
non-steroidal anti-inflammatory agents intermittently. Two
years
previously she had a fibroma removed from her right breast. She is
a non-smoker and drinks
approximately 8 units of alcohol per
week.

Investigations pre-operatively show:

Hb 105 g/L (115-165)

MCV 71 fL (80-96)

WCC 8.4 ×109/L (4-11)

Platelets 401 ×109/L (150-400)

The procedure was uncomplicated and she was given two units of
packed red cells postoperatively. Three days
later she becomes
jaundiced and complains of lassitude.

Investigations post-operatively: Score:

Hb 72 g/L (115-165) Total Answered:


MCV 110 fL (80-96)

WCC 9.5 ×109/L (4-11) Question Navigator

Platelets 395 ×109/L (150-400)


Tags
Which of the following is the best investigation to confirm the
diagnosis?

(Please select 1 option)



Antiglobulin test (indirect)


Direct Coombs' test


Haptoglobin level


Reticulocyte count


Serum iron level

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A 78-year-old lady is admitted to hospital for a right
hemicolectomy for carcinoma of the caecum.
Answer Statistics
She has a history of osteoarthritis for which she takes
non-steroidal anti-inflammatory agents intermittently. Two
years
previously she had a fibroma removed from her right breast. She is
a non-smoker and drinks Test Analysis
approximately 8 units of alcohol per
week.

Investigations pre-operatively show:

Hb 105 g/L (115-165)

MCV 71 fL (80-96)

WCC 8.4 ×109/L (4-11)

Platelets 401 ×109/L (150-400)

The procedure was uncomplicated and she was given two units of
packed red cells postoperatively. Three days
later she becomes
jaundiced and complains of lassitude.

Investigations post-operatively:

Hb 72 g/L (115-165)

MCV 110 fL (80-96)


Score:

WCC 9.5 ×109/L (4-11) Total Answered:

Platelets 395 ×109/L (150-400)


Feedback
Which of the following is the best investigation to confirm the
diagnosis?

(Please select 1 option) Question Navigator


Antiglobulin test (indirect)
Incorrect answer selected
Revision Notes
Direct Coombs' test
This is the correct answer


Haptoglobin level Tags

Reticulocyte count


Serum iron level

The DCT will specifically confirm immune-mediated haemolysis,


such as this transfusion reaction.

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A 32-year-old Afro-Caribbean lady is admitted to hospital with a
three day history of increasing pain and swelling
Test Analysis
of her left leg.
Over the past 24 hours she has also developed a low-grade fever.
There is no history of recent
trauma, immobilisation or prolonged
travel by land or air.

She was admitted two years previously with a similar episode of


pain and swelling in her right calf; a deep vein
thrombosis was
confirmed on Doppler scanning. She is otherwise fit and well. She
is married and lives with her
husband and works on a production
line for a company producing televisions. She is a non-smoker and
does not
drink alcohol.

She had been taking the oral contraceptive pill when admitted
two years ago with a DVT, but this was then
discontinued. Her only
medication is an antidepressant, prescribed by her GP after she had
a miscarriage six
months previously. Her relationship with a
previous partner ended after she miscarried his child.

Investigations showed:

Hb 105 g/L (115-165)

MCV 94 fL (80-96)

7.5 ×109/L Score:


WCC (4-11)
Total Answered:
Platelets 95 ×109/L (150-400)

Protein C Normal activity -


Question Navigator
Protein S Normal activity -

VDRL Positive 1:8 -


Tags
What is the likely diagnosis?

(Please select 1 option)



Antiphospholipid syndrome


Factor V Leiden mutation


Hyperhomocysteinaemia


Pelvic inflammatory disease


Tertiary syphilis

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A 32-year-old Afro-Caribbean lady is admitted to hospital with a
three day history of increasing pain and swelling
Answer Statistics
of her left leg.
Over the past 24 hours she has also developed a low-grade fever.
There is no history of recent
trauma, immobilisation or prolonged
travel by land or air.
Test Analysis
She was admitted two years previously with a similar episode of
pain and swelling in her right calf; a deep vein
thrombosis was
confirmed on Doppler scanning. She is otherwise fit and well. She
is married and lives with her
husband and works on a production
line for a company producing televisions. She is a non-smoker and
does not
drink alcohol.

She had been taking the oral contraceptive pill when admitted
two years ago with a DVT, but this was then
discontinued. Her only
medication is an antidepressant, prescribed by her GP after she had
a miscarriage six
months previously. Her relationship with a
previous partner ended after she miscarried his child.

Investigations showed:

Hb 105 g/L (115-165)

MCV 94 fL (80-96)

WCC 7.5 ×109/L (4-11)

Platelets 95 ×109/L (150-400)


Score:
Protein C Normal activity -
Total Answered:
Protein S Normal activity -

VDRL Positive 1:8 - Feedback


What is the likely diagnosis?
Question Navigator
(Please select 1 option)
Antiphospholipid syndrome
Correct
Revision Notes

Factor V Leiden mutation


Hyperhomocysteinaemia Tags

Pelvic inflammatory disease


Tertiary syphilis

Antiphospholipid syndrome is characterised by recurrent venous


and arterial thrombosis, recurrent abortions and
various
neuropsychiatric illnesses.

Laboratory tests show thrombocytopenia, elevated activated


partial thromboplastin time and false positive VDRL.

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A 35-year-old woman with sickle cell disease was admitted to
hospital with abdominal pain.
Test Analysis
This appearance was noted on fundoscopy.

Score:

Total Answered:

Question Navigator

What is the diagnosis? Tags

(Please select 1 option)



Angioid streaks


Branch retinal artery occlusion


Central retinal vein occlusion


Lipaemia retinalis


Papilloedema

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A 35-year-old woman with sickle cell disease was admitted to
hospital with abdominal pain.
Answer Statistics
This appearance was noted on fundoscopy.
Test Analysis

Score:

Total Answered:

What is the diagnosis? Feedback

(Please select 1 option)


Question Navigator
Angioid streaks
Correct


Branch retinal artery occlusion Revision Notes

Central retinal vein occlusion
Tags

Lipaemia retinalis


Papilloedema

The slide shows angioid streaks, caused by breaks in Bruch's


membrane.

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With which of the following diseases is this appearance
associated?
Test Analysis

Score:

Total Answered:

Question Navigator
(Please select 1 option)

Acquired Immune deficiency syndrome Tags

Chronic lymphocytic leukaemia


Hypertriglyceridaemia


Paget's disease of bone


Poorly controlled diabetes mellitus

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With which of the following diseases is this appearance
associated?
Answer Statistics

Test Analysis

Score:

Total Answered:
(Please select 1 option)
Acquired Immune deficiency syndrome
Incorrect answer selected Feedback

Chronic lymphocytic leukaemia
Question Navigator

Hypertriglyceridaemia

Paget's disease of bone


This is the correct answer Revision Notes

Poorly controlled diabetes mellitus
Tags

The slide shows angioid streaks, caused by breaks in Bruch's


membrane.

Angioid streaks may be associated with pseudoxanthoma elasticum,


Ehlers-Danlos syndrome, sickle cell
disease, acromegaly (and other
pituitary disorders), and Paget's disease.

A useful mnemonic for angioid retinal streak is SLAPPERS:


S - Sickle
L - Lead poisoming
A - Abetalipoproteinaemia/acromegaly
P - Pagets/phacomatoses (tuberous, sclerosis,
neurofibromatosis, Sturge-Weber)
P - Pseudoxanthoma elasticum
E - Ehlers-Danlos
R - Raised calcium or phosphate
S - Short people (dwarfism).

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This patient originally presented ten years ago with a pruritic
rash on her abdomen. These lesions now cover her
Test Analysis
entire body.

This disease is caused by infiltration of which of the


following? Score:

(Please select 1 option) Total Answered:



B cells


Eosinophils Question Navigator

Mast cells
Tags

Neutrophils


T cells

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This patient originally presented ten years ago with a pruritic
rash on her abdomen. These lesions now cover her
Answer Statistics
entire body.

Test Analysis

This disease is caused by infiltration of which of the


following?

(Please select 1 option)


B cells
Incorrect answer selected Score:


Eosinophils Total Answered:


Mast cells
Feedback

Neutrophils

T cells
This is the correct answer Question Navigator

Revision Notes
The diagnosis is mycosis fungoides, a cutaneous T cell
lymphoma.

The disease presents as a pruritic eczematous rash - the


pre-malignant stage - and develops telangiectasias and
Tags
areas of
'cigarette paper' atrophy (poikiloderma atrophicans vasculare).

As malignancy develops, nodular lesions appear and proceed to


become necrotic.

Further Reading:

Macmillan Cancer Support. Cutaneous T-cell lymphoma.

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This gentleman first presented in his early 20s with
haemoptysis. He has required intermittent blood transfusions
Test Analysis

throughout his life.

Score:

Total Answered:

Question Navigator

What is the diagnosis? Tags


(Please select 1 option)

Hepatitis C


Hereditary haemorrhagic telangiectasia


Kaposi's sarcoma


Peutz-Jegher's syndrome


Wegener's granulomatosis

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This gentleman first presented in his early 20s with
haemoptysis. He has required intermittent blood transfusions
Answer Statistics

throughout his life.

Test Analysis

Score:

Total Answered:

What is the diagnosis? Feedback


(Please select 1 option)
Hepatitis C
Incorrect answer selected
Question Navigator

Hereditary haemorrhagic telangiectasia


This is the correct answer
Revision Notes

Kaposi's sarcoma


Peutz-Jegher's syndrome Tags

Wegener's granulomatosis

Typical telangiectasia of hereditary haemorrhagic telangiectasia


(also known as Osler-Weber-Rendu syndrome)
are seen.

The condition is inherited as an autosomal dominant trait;


sporadic cases also occur.

Patients typically present early in adult life with anaemia due


to occult bleeding from gastrointestinal
telangiectasiae.

Lesions commonly occur in the mouth and on the face.

Complications of the disease include haemoptysis and pulmonary


haemorrhage.

Further Reading:

HHT Foundation International. Hereditary Hemorrhagic Telangiectasia Summary for


Physicians and Health Care
Providers
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A 73-year-old lady presents with fatigue.
Test Analysis
Investigations show:
Haemoglobin 85 g/L (115-165)

MCV 69 fL (80-96)

White cell count 5.2 ×109/L (4-11)

Platelets 240 ×109/L (150-400)

Physical examination reveals several erythematous lesions on the


tongue:

Score:

Total Answered:

Question Navigator

Tags

She was transfused with four units of packed red cells.


Oesophagogastroduodenoscopy and colonoscopy did not
demonstrate any
other lesions within the gastrointestinal tract.

What is the best course of subsequent management?

(Please select 1 option)



Argon beam ablation therapy


Genetic counselling


Nasal skin graft


Oestrogen therapy


Oral iron therapy

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A 73-year-old lady presents with fatigue.
Answer Statistics
Investigations show:
Haemoglobin 85 g/L (115-165) Test Analysis
MCV 69 fL (80-96)

White cell count 5.2 ×109/L (4-11)

Platelets 240 ×109/L (150-400)

Physical examination reveals several erythematous lesions on the


tongue:

Score:

Total Answered:

Feedback

Question Navigator

Revision Notes

Tags
She was transfused with four units of packed red cells.
Oesophagogastroduodenoscopy and colonoscopy did not
demonstrate any
other lesions within the gastrointestinal tract.

What is the best course of subsequent management?

(Please select 1 option)


Argon beam ablation therapy
Incorrect answer selected


Genetic counselling


Nasal skin graft


Oestrogen therapy

Oral iron therapy


This is the correct answer

The slide shows the typical appearance of hereditary


haemorrhagic telangiectasia (also known as Osler-Weber-
Rendu
disease) - an autosomal dominant condition associated with bleeding
(usually nose and gastrointestinal
tract) from early teens and
worsening after the age of 50. There may also be AV malformations
of the brain, lung
and GI tract.

Argon ablation is used for gastric antral vascular ectasia


(GAVE).1

Nasal skin grafts are used for persistent epistaxis but this
lady has telangiectasia in many other places.

Oestrogen therapy can be effective especially in women but the


evidence is not strong.2

Reference
1. Jabbari M, et al. Gastric antral vascular ectasia: the watermelon
stomach. Gastroenterology.
1984;87(5):1165-70.
2. van Cutsem E, et al. Treatment of bleeding gastrointestinal vascular
malformations with oestrogen-
progesterone. Lancet.
1990;335(8695):953-5.

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A 45-year-old woman presented to the Emergency department with a
high spiking fever ten days after returning
Test Analysis
from holiday in
Kenya.

Before leaving the United Kingdom her general practitioner had


prescribed mefloquine malaria prophylaxis. She
had started the
mefloquine one week before travelling and had continued to take it
while she was in Africa.

She complained of regular spiking fevers and rigors and of


lethargy and malaise. Physical examination was
unremarkable apart
from a temperature of 40°C. A peripheral blood film is shown
below.

Score:

Total Answered:

Question Navigator

Tags
What is the diagnosis?

(Please select 1 option)



African trypanosomiasis


Dengue fever


Loa loa


Onchocerciasis


Plasmodium falciparum malaria

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A 45-year-old woman presented to the Emergency department with a
high spiking fever ten days after returning
Answer Statistics
from holiday in
Kenya.

Before leaving the United Kingdom her general practitioner had


prescribed mefloquine malaria prophylaxis. She Test Analysis
had started the
mefloquine one week before travelling and had continued to take it
while she was in Africa.

She complained of regular spiking fevers and rigors and of


lethargy and malaise. Physical examination was
unremarkable apart
from a temperature of 40°C. A peripheral blood film is shown
below.

Score:

Total Answered:

Feedback
What is the diagnosis?

(Please select 1 option) Question Navigator


African trypanosomiasis
Incorrect answer selected


Dengue fever Revision Notes

Loa loa
Tags

Onchocerciasis

Plasmodium falciparum malaria


This is the correct answer

The incubation peroid for falciparum malaria is approximately 12


days.

Chemoprophylaxis should be started one week before travelling to


a malaria-endemic country and continued
for one month after
returning.

Severe malaria is defined parasitologically as more than 5% of


red blood cells parasitised. However, the degree
of parasitaemia on
the peripheral film is usually a gross underestimate of the true
parasite burden, as only
younger ring forms are seen in the
peripheral circulation in falciparum malaria.

In the slide shown, the blood film shows ring forms within
erythrocytes; some erythrocytes contain two to
three parasites per
cell - typical of falciparum; other forms of malaria seldom have
more than one parasite per
red cell.

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A 17-year-old girl with sickle cell disease presents with pain
in her left thigh.
Test Analysis
A plain x ray film shows changes consistent with osteomyelitis
of the left femur.

Which of the following is the most likely causative


organism?

(Please select 1 option)



Clostridium perfringens


Enterococcus faecium


Listeria monocytogenes


Mycobacterium tuberculosis


Salmonella enteritidis

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Total Answered:

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A 17-year-old girl with sickle cell disease presents with pain
in her left thigh.
Answer Statistics
A plain x ray film shows changes consistent with osteomyelitis
of the left femur.

Which of the following is the most likely causative


organism? Test Analysis

(Please select 1 option)


Clostridium perfringens
Incorrect answer selected


Enterococcus faecium


Listeria monocytogenes


Mycobacterium tuberculosis

Salmonella enteritidis
This is the correct answer

Patients with sickle cell disease have a predisposition to


develop osteomyelitis due to Salmonella species.

Functional hyposplenism in sickle cell disease also renders


sufferers susceptible to infection with encapsulated
bacteria
(pneumococci, meningococci).
Score:

Total Answered:
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A 62-year-old woman undergoes successful mitral valve
replacement (mechanical valve) for rheumatic valvular
Test Analysis
heart
disease.

Post-operatively she is started on warfarin and her INR is


stabilised with a plan to maintain her INR within the
range 3.0 -
4.5.

Six months later she consults her general practitioner,


complaining of recurrent episodes of dysuria and
frequency. Her GP
starts co-trimoxazole.

What action should be taken to ensure that her INR stays in the
target range?

(Please select 1 option)



Increase dose of warfarin


Maintain the same dose of warfarin


Reduce dose of warfarin


Start vitamin K
Score:

Stop warfarin and switch to subcutaneous low molecular weight heparin
Total Answered:

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A 62-year-old woman undergoes successful mitral valve
replacement (mechanical valve) for rheumatic valvular
Answer Statistics
heart
disease.

Post-operatively she is started on warfarin and her INR is


stabilised with a plan to maintain her INR within the Test Analysis
range 3.0 -
4.5.

Six months later she consults her general practitioner,


complaining of recurrent episodes of dysuria and
frequency. Her GP
starts co-trimoxazole.

What action should be taken to ensure that her INR stays in the
target range?

(Please select 1 option)


Increase dose of warfarin
Incorrect answer selected


Maintain the same dose of warfarin

Reduce dose of warfarin


This is the correct answer


Start vitamin K


Stop warfarin and switch to subcutaneous low molecular weight heparin

Score:
Interaction between co-trimoxazole and warfarin results in
enhanced anticoagulant effect.
Total Answered:
The dose of warfarin should be reduced.

Feedback

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A 25-year-old woman undergoing treatment for promyelocytic (M3)
leukaemia develops a high fever.
Test Analysis
Sepsis is suspected and she rapidly develops widespread
ecchymoses and starts bleeding from venepuncture
sites and mucous
membranes.

Which is the most specific laboratory test to confirm the


diagnosis of disseminated intravascular coagulation
(DIC)?

(Please select 1 option)



Activated partial thromoplastin time


D-dimer assay and fibrin degradation products (FDP)


INR


Plasma fibrinogen concentration


Platelet count

Score:

Total Answered:
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A 25-year-old woman undergoing treatment for promyelocytic (M3)
leukaemia develops a high fever.
Answer Statistics
Sepsis is suspected and she rapidly develops widespread
ecchymoses and starts bleeding from venepuncture
sites and mucous
membranes. Test Analysis
Which is the most specific laboratory test to confirm the
diagnosis of disseminated intravascular coagulation
(DIC)?

(Please select 1 option)


Activated partial thromoplastin time
Incorrect answer selected

D-dimer assay and fibrin degradation products (FDP)


This is the correct answer


INR


Plasma fibrinogen concentration


Platelet count

The platelet count and the fibrinogen level are almost always
decreased in the presence of DIC.

Although these tests are not specific for DIC, normal levels
exclude the possibility of DIC. The presence of Score:
schistocytes, or
red cell fragments, is a frequent but non-specific finding in
DIC.
Total Answered:
Increased levels of FDPs occur in a variety of conditions in
which clot formation and lysis occur.

D-dimers are produced by the action of plasmin on cross-linked


fibrin, not by the action of plasmin on unclotted Feedback
fibrinogen.

These tests reflect the microangiopathy of DIC and have been


found to be sensitive, specific, and efficient in the Question Navigator
diagnosis of
DIC.
Revision Notes
In a recent review by Yu and colleagues, the combination of the
D-dimer and the FDP assay provides the most
rapid and specific
diagnosis of DIC.
Tags
References:

Bick RL. Disseminated intravascular coagulation:


pathophysiological mechanisms and manifestations. Semin

Thromb Hemost. 1998;24(1):3-18.

Yu M, et al. Screening tests of disseminated intravascular


coagulation: guidelines for rapid and specific
laboratory
diagnosis. Crit Care Med. 2000;28(6):1777-80.

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A 23-year-old man is noted by his partner to be jaundiced. Two
weeks previously he suffered a mild flu-like
Test Analysis
illness.

His investigations are shown:

Hb 73 g/L (130-180)

MCV 96 fL (80-96)

WCC 8.0 ×109/L (4-11)

Platelets 340 ×109/L (150-400)

Reticulocytes 6.8% (0.5-2.4)

Albumin 41 g/L (37-49)

Total bilirubin 42 µmol/L (1-22)

AST 36 U/L (1-31)

ALP 40 U/L (45-105)


Score:
Urobilinogen ++
Total Answered:
Bilurubin negative
Urine dipstick
Protein negative
Question Navigator
Blood ++

What diagnostic test would be most useful in confirming the


diagnosis? Tags
(Please select 1 option)

Abdominal ultrasound


Cold agglutinin titre


Hepatitis serology


HIV test


Thick blood films

Submit answer
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A 23-year-old man is noted by his partner to be jaundiced. Two
weeks previously he suffered a mild flu-like
Answer Statistics
illness.

His investigations are shown: Test Analysis


Hb 73 g/L (130-180)

MCV 96 fL (80-96)

WCC 8.0 ×109/L (4-11)

Platelets 340 ×109/L (150-400)

Reticulocytes 6.8% (0.5-2.4)

Albumin 41 g/L (37-49)

Total bilirubin 42 µmol/L (1-22)

AST 36 U/L (1-31)

ALP 40 U/L (45-105)

Urobilinogen ++

Bilurubin negative Score:


Urine dipstick
Protein negative
Total Answered:
Blood ++

What diagnostic test would be most useful in confirming the


diagnosis? Feedback
(Please select 1 option)
Question Navigator
Abdominal ultrasound
Incorrect answer selected

Cold agglutinin titre


This is the correct answer
Revision Notes

Hepatitis serology


HIV test Tags


Thick blood films

Secondary cold agglutinin disease typically presents with


anaemia and haemoglobinuria due to intravascular
haemolysis two to
three weeks following infection such as with:
Mycoplasma pneumoniae
Viruses (EBV, CMV, etc)
Legionnaires' disease
Malaria, etc.

Cold agglutinins occur normally but at very low titres.

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A 70-year-old lady presents with recurrent lower respiratory
tract infections.
Test Analysis
She has had three courses of antibiotics from her general
practitioner in the past four months. She has no history
of asthma
or COPD. She denies sputum production between the episodes of
infection.

Investigations reveal the following:

Hb 101 g/L (115-165)

WCC 29 ×109/L (4-11)

Neutrophils 4 ×109/L (1.5-7)

Lymphocytes 22 ×109/L (1.5-4)

Platelets 180 ×109/L (150-400)

The blood film is normochromic and normocytic with evidence of


polychromasia.

Which of the following investigations would provide the most


information?
Score:
(Please select 1 option)
Total Answered:

Acid haemolysis test


Bone marrow aspirate and trephine biopsy
Question Navigator

Direct Coombs' antiglobulin test


Reticulocyte count Tags


Serum immunoglobulins

Submit answer
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A 70-year-old lady presents with recurrent lower respiratory
tract infections.
Answer Statistics
She has had three courses of antibiotics from her general
practitioner in the past four months. She has no history
of asthma
or COPD. She denies sputum production between the episodes of
infection. Test Analysis
Investigations reveal the following:

Hb 101 g/L (115-165)

WCC 29 ×109/L (4-11)

Neutrophils 4 ×109/L (1.5-7)

Lymphocytes 22 ×109/L (1.5-4)

Platelets 180 ×109/L (150-400)

The blood film is normochromic and normocytic with evidence of


polychromasia.

Which of the following investigations would provide the most


information?

(Please select 1 option)


Acid haemolysis test
Incorrect answer selected Score:
Bone marrow aspirate and trephine biopsy
This is the correct answer Total Answered:

Direct Coombs' antiglobulin test


Reticulocyte count Feedback


Serum immunoglobulins
Question Navigator

This lady has chronic lymphocytic leukaemia. Revision Notes


There is a very high white cell count which is predominantly
lymphocytes.
Tags
The key investigations should be aimed at confirming the
diagnosis and looking for potential complications.

The anaemia and recurrent infections suggest the complications


of autoimmune haemolytic anaemia and
hypogammaglobulinaemia.

Polychromasia on blood film suggests a reticulocytosis and, in


the presence of anaemia, supports a diagnosis of
haemolysis.

Immune deficiency may be due to hypogammaglobulinaemia or


myelosuppression from bone marrow infiltration.

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A 28-year-old lady presents with a three day history of a
painful swollen right calf.
Test Analysis
Her coagulation screen shows:

Prothrombin time 13 s (11.5-15.5)

Thrombin time 13 s (13)

Activated partial thromboplastin time 78 s (30-40)

The APTT was not corrected when mixed with normal plasma.

What is the cause of the clotting abnormality?

(Please select 1 option)



Chronic liver disease


Disseminated intravascular coagulation


Haemophilia
Score:

Lupus anticoagulant
Total Answered:

von Willebrand disease

Question Navigator

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A 28-year-old lady presents with a three day history of a
painful swollen right calf.
Answer Statistics
Her coagulation screen shows:

Prothrombin time 13 s (11.5-15.5)


Test Analysis

Thrombin time 13 s (13)

Activated partial thromboplastin time 78 s (30-40)

The APTT was not corrected when mixed with normal plasma.

What is the cause of the clotting abnormality?

(Please select 1 option)


Chronic liver disease
Incorrect answer selected


Disseminated intravascular coagulation


Haemophilia

Lupus anticoagulant
This is the correct answer


von Willebrand disease
Score:

Total Answered:
The abnormality shown is a prolonged APTT.

Chronic liver disease would cause a prolonged prothrombin time


(PT) so this can be excluded immediately. Feedback
All the other options are causes of prolonged APTT but it is
only lupus anticoagulant that does not correct when
mixed with
normal plasma. Question Navigator

Also the clues in the case history are young female and
suspected DVT, which with recurrent miscarriages,
Revision Notes
strokes and
thrombocytopenia, are features of the antiphospholipid
syndrome.

Tags
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You are called to see a 58-year-old lady on the ward because the
nursing staff have noticed the following
Test Analysis
abnormality of her
eye.

She is an inpatient with a history of paroxysmal atrial


fibrillation and rheumatoid arthritis. The lady complains only
of a
minor ache in the eye.

Score:

Total Answered:

Question Navigator

Tags

Which of her medication is a likely cause of the lesion?

(Please select 1 option)



Amiodarone


Aspirin


Folic acid


Meloxicam


Methotrexate

Submit answer
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You are called to see a 58-year-old lady on the ward because the
nursing staff have noticed the following
Answer Statistics
abnormality of her
eye.

She is an inpatient with a history of paroxysmal atrial


fibrillation and rheumatoid arthritis. The lady complains only Test Analysis
of a
minor ache in the eye.

Score:

Total Answered:

Feedback

Question Navigator

Revision Notes
Which of her medication is a likely cause of the lesion?
Tags
(Please select 1 option)
Amiodarone
Incorrect answer selected

Aspirin
This is the correct answer


Folic acid


Meloxicam


Methotrexate

The picture shows a subconjunctival haemorrhage which is an


alarming adverse effect of aspirin therapy (and
other
antiplatelets).

It usually resolves over 10-14 days.

If the haematoma is large it may be worth considering


prophylactic antibiotic eyedrops.

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A 28-year-old plumber was referred to hospital by his general
practitioner.
Test Analysis
He had initially presented seven days previously, giving a three
day history of malaise, headache, and myalgia
and subsequently
developed a dry cough and fever.

His GP had started a course of amoxicillin/clavulinic acid, but


the symptoms failed to resolve. On the day of
referral, the patient
continued to complain of cough and had become mildly dyspnoeic; he
also complained of a
global headache, myalgia and arthralgia.

On examination, he appeared unwell and was febrile (39°C). A


maculopapular rash was evident over his upper
body. Heart sounds
were normal; BP 120/70 mmHg. On auscultation of his chest, fine
crackles were audible in
the left mid-zone. Mild neck stiffness was
noted.

Investigations revealed:

Hb 84 g/L (130-180)

WBC 8 ×109/L (4-11)

Platelets 210 ×109/L (150-400) Score:

Reticulocytes 8% (0.5-2.4) Total Answered:


 

Na 129 mmol/L (137-144) Question Navigator


K 4.2 mmol/L (3.5-4.9)

Urea 5.0 mmol/L (2.5-7.5)


Tags

Creatinine 110 µmol/L (60-110)

Bilirubin 19 µmol/L (1-22)

Alk phos 130 U/L (45-105)

AST 54 U/L (1-31)

GGT 48 U/L (<50)

Chest x ray shows patchy consolidation in both mid-zones.

Which investigation should be performed to confirm the cause of


his abnormal blood count?

(Please select 1 option)



Bone marrow aspirate


Direct Coombs' test


Hepatitis serology


Paul-Bunnell test


Unconjugated bilirubin

Submit answer
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A 28-year-old plumber was referred to hospital by his general
practitioner.
Answer Statistics
He had initially presented seven days previously, giving a three
day history of malaise, headache, and myalgia
and subsequently
developed a dry cough and fever. Test Analysis
His GP had started a course of amoxicillin/clavulinic acid, but
the symptoms failed to resolve. On the day of
referral, the patient
continued to complain of cough and had become mildly dyspnoeic; he
also complained of a
global headache, myalgia and arthralgia.

On examination, he appeared unwell and was febrile (39°C). A


maculopapular rash was evident over his upper
body. Heart sounds
were normal; BP 120/70 mmHg. On auscultation of his chest, fine
crackles were audible in
the left mid-zone. Mild neck stiffness was
noted.

Investigations revealed:

Hb 84 g/L (130-180)

WBC 8 ×109/L (4-11)

Platelets 210 ×109/L (150-400)

Reticulocytes 8% (0.5-2.4)
Score:
 

Na 129 mmol/L (137-144) Total Answered:

K 4.2 mmol/L (3.5-4.9)

Urea 5.0 mmol/L (2.5-7.5)


Feedback

Creatinine 110 µmol/L (60-110)


Question Navigator
 

Bilirubin 19 µmol/L (1-22)


Revision Notes
Alk phos 130 U/L (45-105)

AST 54 U/L (1-31) Tags


GGT 48 U/L (<50)

Chest x ray shows patchy consolidation in both mid-zones.

Which investigation should be performed to confirm the cause of


his abnormal blood count?

(Please select 1 option)


Bone marrow aspirate
Incorrect answer selected

Direct Coombs' test


This is the correct answer


Hepatitis serology


Paul-Bunnell test


Unconjugated bilirubin

The presentation is typical of Mycoplasma pneumonia:


pneumonia associated with mild hepatitis and haemolytic

anaemia.
Extrapulmonary manifestations occur in ~10% of cases:
Haemolytic anaemia
Renal failure
Hepatitis
Myocarditis
Meningism
Meningitis
Transverse myelitis, or
Cerebellar ataxia.

Cutaneous manifestations include erythema multiforme.

Haemolysis is associated with the presence of IgM antibodies


(cold agglutinins) directed against the I antigen of
the
erythrocyte membrane. Haemolysis would be confirmed on a blood film
and a direct Coombs' test.

Unconjugated bilirubin would support haemolysis, but is not a


diagnostic test.

Infectious mononucleosis may also present with haemolytic


anaemia.

However, the presentation is not consistent with acute


Epstein-Barr virus.

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A 72-year-old man is referred to clinic with a four month
history of breathlessness and abdominal swelling and
Test Analysis

discomfort.

On examination, he appears pale and unwell. His liver is


palpable 12 cm below the right costal margin and his
spleen
palpable 15 cm below the left costal margin. No lymphadenopathy was
detected.

Investigations revealed:
Hb 59 g/L (130-180)

RBC 2.1 ×1012/L -

PCV 0.17 l/l -

MCH 30 pg (28-32)

MCV 82 fL (80-96)

Reticulocytes 1.4% (0.5-2.4)

Total WBC 23 ×109/L (4-11)

Normoblasts 8% - Score:

Platelets 280 ×109/L (150-400) Total Answered:

 
Question Navigator
Neutrophils 9.0 ×109/L (1.5-7)

Lymphocytes 5.2 ×109/L (1.5-4) Tags


Monocytes 1.3 ×109/L (0-0.8)

Eosinophils 0.2 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1)

Metamyelocytes 5.1 ×109/L -

Myelocytes 1.6 ×109/L -

Blast cells 0.4 ×109/L -

Blood film: Anisocytosis, poikilocytosis and occasional


erythrocyte tear drop cells.

What is the correct name given to this blood picture?

(Please select 1 option)



Anaemia of chronic disease


Blastic transformation


Leukemoid reaction


Leukoerythroblastic anaemia


Myeloid leukaemia


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A 72-year-old man is referred to clinic with a four month
history of breathlessness and abdominal swelling and
Answer Statistics

discomfort.

On examination, he appears pale and unwell. His liver is


palpable 12 cm below the right costal margin and his Test Analysis
spleen
palpable 15 cm below the left costal margin. No lymphadenopathy was
detected.

Investigations revealed:
Hb 59 g/L (130-180)

RBC 2.1 ×1012/L -

PCV 0.17 l/l -

MCH 30 pg (28-32)

MCV 82 fL (80-96)

Reticulocytes 1.4% (0.5-2.4)

Total WBC 23 ×109/L (4-11)

Normoblasts 8% -

Platelets 280 ×109/L (150-400)


Score:
 
Total Answered:
Neutrophils 9.0 ×109/L (1.5-7)

Lymphocytes 5.2 ×109/L (1.5-4) Feedback


Monocytes 1.3 ×109/L (0-0.8)
Question Navigator
Eosinophils 0.2 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1) Revision Notes


Metamyelocytes 5.1 ×109/L -
Tags
Myelocytes 1.6 ×109/L -

Blast cells 0.4 ×109/L -

Blood film: Anisocytosis, poikilocytosis and occasional


erythrocyte tear drop cells.

What is the correct name given to this blood picture?

(Please select 1 option)


Anaemia of chronic disease
Incorrect answer selected


Blastic transformation


Leukemoid reaction

Leukoerythroblastic anaemia
This is the correct answer


Myeloid leukaemia

In leukoerythroblastic reactions the peripheral blood contains


immature white cells and nucleated red cells
irrespective of the
total white cell count and circulating blasts may be seen.
The finding of tear drop cells is characteristic of
myelofibrosis.

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A 72-year-old man is referred to clinic with a four month
history of breathlessness, abdominal swelling and
Test Analysis
discomfort.

On examination, he appears pale and unwell. His liver is


palpable 9 cm below the right costal margin and his
spleen palpable
7 cm below the left costal margin. No lymphadenopathy was
detected.

Investigations show:

Hb 79 g/L (130-180)

RBC 2.1 ×1012/L -

PCV 0.17 -

MCH 30 pg (28-32)

MCV 82 fL (80-96)

Reticulocytes 1.4% (0.5-2.4)

Total WBC 23 ×109/L (4-11)


Score:
Normoblasts 8% -
Total Answered:
Platelets 280 ×109/L (150-400)

 
Question Navigator
Neutrophils 9.0 ×109/L (1.5-7)

Lymphocytes 5.2 ×109/L (1.5-4) Tags

Monocytes 1.3 ×109/L (0-0.8)

Eosinophils 0.2 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1)

Metamyelocytes 5.1 ×109/L -

Myelocytes 1.6 ×109/L -

Blast cells 0.4 ×109/L -

Blood film: Anisocytosis, poikilocytosis and occasional


erythrocyte tear drop cells.

What is the first investigation of choice?

(Please select 1 option)



Bone marrow culture for mycobacteria


Bone marrow smear


Bone scan


Bone (trephine) biopsy


Leucocyte alkaline phosphatase

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A 72-year-old man is referred to clinic with a four month
history of breathlessness, abdominal swelling and
Answer Statistics
discomfort.

On examination, he appears pale and unwell. His liver is


palpable 9 cm below the right costal margin and his Test Analysis
spleen palpable
7 cm below the left costal margin. No lymphadenopathy was
detected.

Investigations show:

Hb 79 g/L (130-180)

RBC 2.1 ×1012/L -

PCV 0.17 -

MCH 30 pg (28-32)

MCV 82 fL (80-96)

Reticulocytes 1.4% (0.5-2.4)

Total WBC 23 ×109/L (4-11)

Normoblasts 8% -

Platelets 280 ×109/L (150-400) Score:


  Total Answered:
Neutrophils 9.0 ×109/L (1.5-7)

Lymphocytes 5.2 ×109/L (1.5-4) Feedback

Monocytes 1.3 ×109/L (0-0.8)


Question Navigator
Eosinophils 0.2 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1) Revision Notes

Metamyelocytes 5.1 ×109/L -


Tags
Myelocytes 1.6 ×109/L -

Blast cells 0.4 ×109/L -

Blood film: Anisocytosis, poikilocytosis and occasional


erythrocyte tear drop cells.

What is the first investigation of choice?

(Please select 1 option)


Bone marrow culture for mycobacteria
Incorrect answer selected


Bone marrow smear


Bone scan

Bone (trephine) biopsy


This is the correct answer


Leucocyte alkaline phosphatase

Trephine biopsy is the preferred initial investigation.


The likely diagnosis is myelofibrosis, a disease characterised
by bone marrow infiltration by fibrous tissue.

As a result, bone marrow aspiration often results in a dry


tap.

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A 70-year-old woman is admitted to hospital with a two month
history of increasing fatigue.
Test Analysis
She has a past history of stable exertional angina, but over the
past few weeks she has also noted that her
episodes of angina have
been occurring with less strenuous physical exertion and has also
noted that her
ankles have become swollen.

Ten years previously she underwent an aortic valve replacement


with a mechanical valve for rheumatic valvular
heart disease; she
also had two coronary artery vein grafts during the same procedure
since pre-operative
angiography had shown significant coronary
artery disease. The valve was functioning normally on transthoracic

echocardiography when she was last reviewed in the cardiology


outpatient clinic three months ago. Her regular
medications consist
of atenolol, warfarin and sublingual nitrate spray.

On examination she appeared pale. She was afebrile. Her pulse


was 90/minute, BP 185/100 mmHg. Her apex
beat was displaced
laterally to the sixth intercostal space but her heart sounds were
normal and lungs were
clear on auscultation. There was bilateral
pitting oedema of the ankles. Her abdomen was soft and non-tender.

The liver edge was palpable 4 cm below the right costal margin.

Investigations revealed:
Score:
Hb 74 g/L (115-165) Total Answered:
WBC 5.2 ×109/L (4-11)

Platelets 189 ×109/L (150-400) Question Navigator

MCV 92 fL (80-96)
Tags
 

Sodium 139 mmol/L (137-144)

Potassium 4.4 mmol/L (3.5-4.9)

Urea 19 mmol/L (2.5-7.5)

Creatinine 376 µmol/L (60-110)

Total protein 96 g/L (61-76)

Albumin 34 g/L (37-49)

Urinalysis: protein +

Blood film: normal appearing red cell morphology; rouleaux


formation noted.

Which investigation would be most helpful in establishing the


diagnosis?

(Please select 1 option)



Abdominal ultrasound scan


Bone marrow aspiration


ESR


Exercise tolerance test


Renal biopsy

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A 70-year-old woman is admitted to hospital with a two month
history of increasing fatigue.
Answer Statistics
She has a past history of stable exertional angina, but over the
past few weeks she has also noted that her
episodes of angina have
been occurring with less strenuous physical exertion and has also
noted that her Test Analysis
ankles have become swollen.

Ten years previously she underwent an aortic valve replacement


with a mechanical valve for rheumatic valvular
heart disease; she
also had two coronary artery vein grafts during the same procedure
since pre-operative
angiography had shown significant coronary
artery disease. The valve was functioning normally on transthoracic

echocardiography when she was last reviewed in the cardiology


outpatient clinic three months ago. Her regular
medications consist
of atenolol, warfarin and sublingual nitrate spray.

On examination she appeared pale. She was afebrile. Her pulse


was 90/minute, BP 185/100 mmHg. Her apex
beat was displaced
laterally to the sixth intercostal space but her heart sounds were
normal and lungs were
clear on auscultation. There was bilateral
pitting oedema of the ankles. Her abdomen was soft and non-tender.

The liver edge was palpable 4 cm below the right costal margin.

Investigations revealed:

Hb 74 g/L (115-165)

WBC 5.2 ×109/L (4-11)


Score:

189 ×109/L Total Answered:


Platelets (150-400)

MCV 92 fL (80-96)
Feedback
 

Sodium 139 mmol/L (137-144)


Question Navigator
Potassium 4.4 mmol/L (3.5-4.9)

Urea 19 mmol/L (2.5-7.5) Revision Notes


Creatinine 376 µmol/L (60-110)

Total protein 96 g/L (61-76) Tags


Albumin 34 g/L (37-49)

Urinalysis: protein +

Blood film: normal appearing red cell morphology; rouleaux


formation noted.

Which investigation would be most helpful in establishing the


diagnosis?

(Please select 1 option)


Abdominal ultrasound scan
Incorrect answer selected

Bone marrow aspiration


This is the correct answer


ESR


Exercise tolerance test


Renal biopsy

The most likely diagnosis is multiple myeloma.


The globulin level is markedly raised (albumin + globulin =
total protein), suggesting the presence of a
paraprotein.

Multiple myeloma may present with roleaux formation on blood


film and raised total protein (globulin component).

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This blood film was obtained on a 37-year-old male.
Test Analysis

Score:

What is the likely cause for these abnormalities? Total Answered:

(Please select 1 option)



Disseminated intravascular coagulation Question Navigator


Hereditary spherocytosis
Tags

Malaria


Splenectomy


Trypanosmiasis

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This blood film was obtained on a 37-year-old male.
Answer Statistics

Test Analysis

What is the likely cause for these abnormalities?


Score:
(Please select 1 option)
Total Answered:
Disseminated intravascular coagulation
Incorrect answer selected


Hereditary spherocytosis
Feedback

Malaria

Splenectomy
This is the correct answer Question Navigator

Trypanosmiasis
Revision Notes

The image shows Howell-Jolly bodies. Tags


These are spherical bluish inclusions within erythrocytes and
are nuclear fragments of condensed DNA which
are normally removed
by the spleen.

They are seen in severe haemolytic anaemias or in


hyposplenic/asplenic patients.

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Work Smart MRCP Part 2 Written 2002 July

A 45-year-old lady with chronic renal failure secondary to


systemic lupus erythematosus is seen in a low
Test Analysis
clearance clinic as a
routine follow up.

Her Joints have been causing some discomfort and she has been
taking naproxen PRN as well as prednisolone
2.5 mg od with
azathioprine 50 mg od for disease control.

She has stable renal function with a creatinine of 300 mmol/l


and a creatinine clearance of 18 ml/min. Associated
with her
chronic renal failure she has controlled secondary
hyperparathyroidism. She has been anaemic for the
last six months
and investigation of dyspepsia with an OGD showed only mild
gastritis. She has already been on
oral ferrous sulphate 200 mg tds
for three months.

Investigations show:
Haemoglobin 94 g/L (115 - 165)

% Hypochromic red cells 12% -

Platelets 180 ×109/L (150 - 400)

White cell count 6.4 ×109/L (4 - 11)


Score:
Serum folate 4.0 ug/L (2 - 11)
Total Answered:
Serum ferritin 230 ug/L (15 - 300)

Transferrin saturation 17% -


Question Navigator
What therapeutic intervention should now be considered?

(Please select 1 option)


Tags

Folate


GM-CSF


Intravenous iron


Subcutaneous erythropoetin


Vitamin B12

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Work Smart MRCP Part 2 Written 2002 July

A 45-year-old lady with chronic renal failure secondary to


systemic lupus erythematosus is seen in a low
Answer Statistics
clearance clinic as a
routine follow up.

Her Joints have been causing some discomfort and she has been
taking naproxen PRN as well as prednisolone Test Analysis
2.5 mg od with
azathioprine 50 mg od for disease control.

She has stable renal function with a creatinine of 300 mmol/l


and a creatinine clearance of 18 ml/min. Associated
with her
chronic renal failure she has controlled secondary
hyperparathyroidism. She has been anaemic for the
last six months
and investigation of dyspepsia with an OGD showed only mild
gastritis. She has already been on
oral ferrous sulphate 200 mg tds
for three months.

Investigations show:
Haemoglobin 94 g/L (115 - 165)

% Hypochromic red cells 12% -

Platelets 180 ×109/L (150 - 400)

White cell count 6.4 ×109/L (4 - 11)

Serum folate 4.0 ug/L (2 - 11)

Serum ferritin 230 ug/L (15 - 300) Score:


Transferrin saturation 17% -
Total Answered:

What therapeutic intervention should now be considered?

(Please select 1 option)


Feedback
Folate
Incorrect answer selected
Question Navigator

GM-CSF

Intravenous iron
This is the correct answer Revision Notes

Subcutaneous erythropoetin
Tags

Vitamin B12

Intravenous iron is the first intervention as her percentage of


hypochromic red cells is 12% and the normal ferritin
represents an
acute phase protein.

She has not responded to oral iron.

Erythropoietin would be a valid alternative but only once the


iron deficiency is corrected. You would check a B12
level.

The National Kidney Foundation have produced Guidelines and Commentaries that state:

"Iron is also essential for hemoglobin formation. The iron


status of the patient with chronic Kidney Disease (CKD)
must be
assessed and adequate iron stores."

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A 60-year-old female of Greek origin is admitted with a two week
history of deteriorating weakness, productive
Test Analysis
cough and
dyspnoea.

She has recently started amoxicillin prescribed by her GP. She


is a smoker of 5 cigarettes per day.

Investigations reveal:

Haemoglobin 92 g/L (115-165)

MCV 118 fL (80-96)

Platelets 372 ×109/L (150-400)

White cell count 9.2 ×109/L (4-11)

Her peripheral blood film is shown below.

Score:

Total Answered:

Question Navigator

Tags

What is the most likely diagnosis?

(Please select 1 option)



Beta-thalassaemia


Glucose-6-phosphate dehydrogenase deficiency


Hypothyroidism


Mycoplasma pneumoniae infection


Lung cancer

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A 60-year-old female of Greek origin is admitted with a two week
history of deteriorating weakness, productive
Answer Statistics
cough and
dyspnoea.

She has recently started amoxicillin prescribed by her GP. She


is a smoker of 5 cigarettes per day. Test Analysis
Investigations reveal:

Haemoglobin 92 g/L (115-165)

MCV 118 fL (80-96)

Platelets 372 ×109/L (150-400)

White cell count 9.2 ×109/L (4-11)

Her peripheral blood film is shown below.

Score:

Total Answered:

Feedback

Question Navigator

Revision Notes

Tags
What is the most likely diagnosis?

(Please select 1 option)


Beta-thalassaemia
Incorrect answer selected


Glucose-6-phosphate dehydrogenase deficiency


Hypothyroidism

Mycoplasma pneumoniae infection


This is the correct answer


Lung cancer

The blood film shows red cell agglutination, suggesting the


presence of cold agglutinin which is associated with
Lymphoma (autoantibodies with anti-I specificity)
Mycoplasma pneumonia (autoantibodies with anti-I
specificity)

and, rarely
Infectious mononucleosis (autoantibodies with anti-I
specificity).
Other laboratory findings in cold agglutinin disease are similar
to those in warm autoimmune haemolytic anaemia
(red blood cell
[RBC] polychromasia, unconjugated bilirubin, haptoglobin, and
haemoglobinuria).

C3 is detected on the RBC surface by the direct antiglobulin


test (DAT).

Cold agglutinin disease with RBC agglutination may be associated


with Raynaud's phenomenon.

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A 23-year-old Afro-Caribbean male presented to the emergency
department with intermittent right upper
Test Analysis
abdominal pain. He had a
temperature of 38.5°C and he was icteric.

Investigations showed:

Haemoglobin 112 g/L (130-180)

Haematocrit 0.36 (0.40-0.52)

MCV 78 fL (80-96)

White cell count 10.2 ×109/L (4-11)

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags

What is the diagnosis?

(Please select 1 option)



Autoimmune haemolytic anaemia


Glucose 6-phosphate dehydrogenase deficiency


Microangiopathic haemolytic anaemia


Pyruvate kinase deficiency


Sickle cell disease

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A 23-year-old Afro-Caribbean male presented to the emergency
department with intermittent right upper
Answer Statistics
abdominal pain. He had a
temperature of 38.5°C and he was icteric.

Investigations showed: Test Analysis


Haemoglobin 112 g/L (130-180)

Haematocrit 0.36 (0.40-0.52)

MCV 78 fL (80-96)

White cell count 10.2 ×109/L (4-11)

His blood film is shown below:

Score:

Total Answered:

Feedback

Question Navigator

What is the diagnosis?


Revision Notes
(Please select 1 option)
Autoimmune haemolytic anaemia
Incorrect answer selected Tags

Glucose 6-phosphate dehydrogenase deficiency


Microangiopathic haemolytic anaemia


Pyruvate kinase deficiency

Sickle cell disease


This is the correct answer

This patient has sickle cell anaemia as evidenced by sickle


cells (not seen in sickle cell trait [HbSC] or
haemoglobin C [HbC]
disease).

Howell-Jolly bodies are also present, indicating


hyposplenism.

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A 33-year-old female with a history of paranoid delusions of one
month duration was treated with phenothiazine.
Test Analysis
She presents with a 10 day history of fever, chills, and
malaise, with increasing weakness and fatigue over the
last two
days.

Examination reveals a temperature of 38.1°C, a pulse of 100


beats per minute regular and a blood pressure of
110/76 mmHg. She
has a respiratory rate of 25/min and chest with dullness to
percussion and decreased breath
sounds at the left base. There is
no splenomegaly.

Investigations reveal:

Haemoglobin 102 g/L (115-165)

Haematocrit 0.384 (0.36-0.47)

MCV 90 fL (80-96)

White cell count 0.9 ×109/L (4-11)

Neutrophils 0.3 ×109/L (1.5-7)


Score:
Lymphocytes 0.3 ×109/L (1.5-4)
Total Answered:
Monocytes 0.01 ×109/L (0-0.8)

Eosinophils 0.01 ×109/L (0.04-0.4) Question Navigator


Platelets 210 ×109/L (150-400)
Tags
 

Serum sodium 131 mmol/L (137-144)

Serum potassium 3.3 mmol/L (3.5-4.9)

Serum urea 4.2 mmol/L (2.5-7.5)

Serum glucose 5.1 mmol/L (3.0-6.0)

The chest x ray shows evidence of left basal consolidation.

What is the most likely cause of her haematological indices?

(Please select 1 option)



Accelerated destruction of neutrophils


Aplastic anaemia


Drug induced


Ineffective erythropoiesis


Shift from circulating pool to marginal granulocyte pool

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Work Smart
A 33-year-old female with a history of paranoid delusions of one
month duration was treated with phenothiazine.
Answer Statistics
She presents with a 10 day history of fever, chills, and
malaise, with increasing weakness and fatigue over the
last two
days. Test Analysis
Examination reveals a temperature of 38.1°C, a pulse of 100
beats per minute regular and a blood pressure of
110/76 mmHg. She
has a respiratory rate of 25/min and chest with dullness to
percussion and decreased breath
sounds at the left base. There is
no splenomegaly.

Investigations reveal:

Haemoglobin 102 g/L (115-165)

Haematocrit 0.384 (0.36-0.47)

MCV 90 fL (80-96)

White cell count 0.9 ×109/L (4-11)

Neutrophils 0.3 ×109/L (1.5-7)

Lymphocytes 0.3 ×109/L (1.5-4)

Monocytes 0.01 ×109/L (0-0.8) Score:

Eosinophils 0.01 ×109/L (0.04-0.4) Total Answered:

Platelets 210 ×109/L (150-400)


Feedback
 

Serum sodium 131 mmol/L (137-144)


Question Navigator
Serum potassium 3.3 mmol/L (3.5-4.9)

Serum urea 4.2 mmol/L (2.5-7.5) Revision Notes


Serum glucose 5.1 mmol/L (3.0-6.0)

The chest x ray shows evidence of left basal consolidation.


Tags

What is the most likely cause of her haematological indices?

(Please select 1 option)


Accelerated destruction of neutrophils
Incorrect answer selected


Aplastic anaemia

Drug induced
This is the correct answer


Ineffective erythropoiesis


Shift from circulating pool to marginal granulocyte pool

This patient has a marked leukopenia with neutropenia and


consequent pneumonia. The most likely explanation
given the history
of phenothiazine use is a drug induced neutropenia.

Phenothiazines are thought to interfere with DNA synthesis and


thus decrease marrow production.
Chemotheraputic agents also
decrease production - killing marrow cells by cytotoxic action.
Aplastic anaemia is unlikely as her haematocrit and platelet
counts are normal so the defect seems confined to
the
leukocytes.

There is no evidence of ineffective hematopoiesis such as


megaloblastic anaemia in that the red blood cells were
of normal
size.

While accelerated destruction of neutrophils may occur in the


setting of pneumonia, the pneumonia seems to
follow and be
secondary to the lack of neutrophils.

Shifts from the circulating pool to the marginal granulocyte


pool may cause decreases in the circulating PB
neutrophils, but
they are usually transient.

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A 27-year-old Afro-Caribbean male attends for a routine
insurance examination.
Test Analysis
Questioning reveals that he has been frequently tired of late
and sleeps in the evenings. He also gives a history
of bleeding
gums and several recent episodes of epitaxis. He is a non-smoker
but drinks 22 units of alcohol
weekly.

Examination reveals a pale appearance but no other


abnormalities.

Investigations show:

Haemoglobin 80 g/L (130-180)

Haematocrit 0.24 (0.40-0.52)

MCV 88 fL (80-96)

White cell count 2 ×109/L (4-11)

Neutrophils 20% -

Lymphocytes 77% -
Score:
Platelets 40 ×109/L (150-400)
Total Answered:
The peripheral blood film showed normocytic hypochromic
erythrocytes.

What is the most likely diagnosis? Question Navigator


(Please select 1 option)
Tags

Accelerated destruction of neutrophils


Aplastic anaemia


Drug induced


Sepsis


Sickle cell crisis

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Work Smart
A 27-year-old Afro-Caribbean male attends for a routine
insurance examination.
Answer Statistics
Questioning reveals that he has been frequently tired of late
and sleeps in the evenings. He also gives a history
of bleeding
gums and several recent episodes of epitaxis. He is a non-smoker
but drinks 22 units of alcohol Test Analysis
weekly.

Examination reveals a pale appearance but no other


abnormalities.

Investigations show:

Haemoglobin 80 g/L (130-180)

Haematocrit 0.24 (0.40-0.52)

MCV 88 fL (80-96)

White cell count 2 ×109/L (4-11)

Neutrophils 20% -

Lymphocytes 77% -

Platelets 40 ×109/L (150-400)

The peripheral blood film showed normocytic hypochromic


erythrocytes. Score:

What is the most likely diagnosis? Total Answered:

(Please select 1 option)


Feedback
Accelerated destruction of neutrophils
Incorrect answer selected

Aplastic anaemia
This is the correct answer
Question Navigator

Drug induced


Sepsis Revision Notes


Sickle cell crisis
Tags

This patient has aplastic anaemia with all three cell lines
severely depressed as evidenced by the low
haematocrit, the low
white count (especially the neutropenia), and the low number of
platelets.

The patient has no features of sepsis and modest alcohol


consumption would not cause this.

The normal smear plus reduced white cell count and platelets
argue against a sickling crisis.

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This peripheral blood smear is obtained on a 16-year-old female
with Down syndrome who is admitted with
Test Analysis
abdominal cramps and
constipation:

Investigations reveal:

Haemoglobin 98 g/L (115-165)

Platelet count 210 ×109/L (150-400)

White cell count 5.5 ×109/L (4-11) Score:

Which of the following would account for her presentation and


appearance of the blood film? Total Answered:

(Please select 1 option)



Acute intermittent porphyria
Question Navigator


Acute lymphoblastic leukaemia
Tags

Aplastic anaemia


Lead poisoning


Splenic infarction

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This peripheral blood smear is obtained on a 16-year-old female
with Down syndrome who is admitted with
Answer Statistics
abdominal cramps and
constipation:

Test Analysis

Investigations reveal:

Haemoglobin 98 g/L (115-165)

Platelet count 210 ×109/L (150-400)

White cell count 5.5 ×109/L (4-11)

Which of the following would account for her presentation and


appearance of the blood film?
Score:
(Please select 1 option)
Total Answered:
Acute intermittent porphyria
Incorrect answer selected


Acute lymphoblastic leukaemia
Feedback

Aplastic anaemia

Lead poisoning
This is the correct answer Question Navigator

Splenic infarction
Revision Notes

The blood film shows basophilic (blue) stippling of the red


cells which is typical of lead poisoning. Tags
This occurs due to accumulation of ribonucleic acid (RNA) in the
red blood cells due to inhibition of pyrimidine 5
nucleotidase by
lead. The denatured RNA is revealed as basophilic stippling.

Other features include mild anaemia, encephalopathy and


peripheral motor neuronopathies.

Patients with learning disabilities may be prone to lead


poisoning due to pica.

Other haematological disorders associated with Down's


syndrome include Fanconi's anaemia, aplasia and acute

myelogenous leukaemia (AML)/acute lymphoblastic leukaemia


(ALL).

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What may be the cause of the appearance of this blood film?
Test Analysis

Score:

Total Answered:

Question Navigator
(Please select 1 option)

Cryoglobulinaemia
Tags

Glandular fever


Hyposplenism


Multiple myeloma


Vitamin C deficiency

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What may be the cause of the appearance of this blood film?
Answer Statistics

Test Analysis

Score:

Total Answered:
(Please select 1 option)
Cryoglobulinaemia
Incorrect answer selected
Feedback

Glandular fever

Hyposplenism
This is the correct answer Question Navigator

Multiple myeloma
Revision Notes

Vitamin C deficiency

Tags
The film shows target cells which are typically found in
hyposplenism.

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A 17-year-old man from India presents with a 12 hour history of
priapism.
Test Analysis
He has a history of intermittent back and abdominal pain. On
examination he is pyrexial 39°C appears pale. He
has an enlarged
smooth liver and a painful engorged penis.

Investigations show:

Haemoglobin 85 g/L (130-180)

MCV 81 fL (80-96)

White cell count 12.2 ×109/L (4-11)

Platelets 450 ×109/L (150-400)

What is the most likely diagnosis?

(Please select 1 option)



Beta thalassaemia major


Idiopathic
Score:


Leukaemia
Total Answered:


Lymphoma
Question Navigator

Sickle cell anaemia

Tags

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Work Smart
A 17-year-old man from India presents with a 12 hour history of
priapism.
Answer Statistics
He has a history of intermittent back and abdominal pain. On
examination he is pyrexial 39°C appears pale. He
has an enlarged
smooth liver and a painful engorged penis. Test Analysis
Investigations show:

Haemoglobin 85 g/L (130-180)

MCV 81 fL (80-96)

White cell count 12.2 ×109/L (4-11)

Platelets 450 ×109/L (150-400)

What is the most likely diagnosis?

(Please select 1 option)


Beta thalassaemia major
Incorrect answer selected


Idiopathic


Leukaemia
Score:

Lymphoma
Total Answered:
Sickle cell anaemia
This is the correct answer

Feedback
Priapism is most often due to idiopathic thrombosis of the
prostatic venous plexus.
Question Navigator
Other causes include leukaemia, sickle-cell anaemia and
carcinomatosis.

Sickle-cell anaemia occurs most frequently in African blacks,


and sporadically in Mediterranean, Middle-East and Revision Notes
India. It may
present with occlusive sickling crises which may start as vague
pain in back, abdomen or long
bones.
Tags
Priapism occurs fairly frequently which may lead to permanent
impotence if it is not relieved.

The elevated WBC reflects stress response rather than anything


more sinister.

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Work Smart MRCP Part 2 Written 2001 July

A 42-year-old widow presents with a one week history of


progressive confusion and unsteady gait. She works as
Test Analysis
a barmaid and
lives in poor social circumstances.

On examination she is malnourished and disorientated. She has


nystagmus and is unable to abduct either eye.
The pupils are
sluggish and unequal. Ankle jerks are absent but upper limb
reflexes are present.

Shortly after her admission you are called to the ward as she
has become very drowsy and has collapsed on the
floor.

Investigations on admission showed:

Haemoglobin 114 g/L (115-165)

MCV 99 fL (80-96)

White blood cells 5.6 ×109/L (4-11)

Platelets 230 ×109/L (150-400)

Serum sodium 129 mmol/L (137-144)


Score:
Serum potassium 3.2 mmol/L (3.5-4.9)
Total Answered:
Serum bilirubin 27 µmol/L (1-22)

Serum gamma glutamyl transferase 440 U/L (4-35)


Question Navigator
Serum alkaline phosphatase 180 U/L (45-105)

Serum aspartate aminotransferase 90 U/L (1-31)


Tags
Serum alanine aminotransferase 45 U/L (5-35)

Serum albumin 33 g/L (37-49)

Prothrombin time 12 secs (11.5-15.5)

Which of the following should be the first investigation?

(Please select 1 option)



B12 and folate levels


Blood cultures


Blood glucose


CT head


EEG

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Work Smart MRCP Part 2 Written 2001 July

A 42-year-old widow presents with a one week history of


progressive confusion and unsteady gait. She works as
Answer Statistics
a barmaid and
lives in poor social circumstances.

On examination she is malnourished and disorientated. She has


nystagmus and is unable to abduct either eye. Test Analysis
The pupils are
sluggish and unequal. Ankle jerks are absent but upper limb
reflexes are present.

Shortly after her admission you are called to the ward as she
has become very drowsy and has collapsed on the
floor.

Investigations on admission showed:

Haemoglobin 114 g/L (115-165)

MCV 99 fL (80-96)

White blood cells 5.6 ×109/L (4-11)

Platelets 230 ×109/L (150-400)

Serum sodium 129 mmol/L (137-144)

Serum potassium 3.2 mmol/L (3.5-4.9)

Serum bilirubin 27 µmol/L (1-22)


Score:
Serum gamma glutamyl transferase 440 U/L (4-35)
Total Answered:
Serum alkaline phosphatase 180 U/L (45-105)

Serum aspartate aminotransferase 90 U/L (1-31)


Feedback
Serum alanine aminotransferase 45 U/L (5-35)

Serum albumin 33 g/L (37-49) Question Navigator


Prothrombin time 12 secs (11.5-15.5)

Revision Notes
Which of the following should be the first investigation?

(Please select 1 option) Tags


B12 and folate levels
Incorrect answer selected


Blood cultures

Blood glucose
This is the correct answer


CT head


EEG

Wernicke's encephalopathy is the most likely diagnosis given


the history and examination findings on a
background of likely
alcoholism.

Although all of the above investigations may need to be


considered to exclude other contributing aetiologies to
her
presentation only the blood glucose can be done quickly and cheaply
at the bedside.

Hypoglycaemia may have precipitated her deterioration and a


finger-prick blood glucose estimation should be
the first
investigation.
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Work Smart MRCP Part 2 Written 2003 December

A 58-year-old lady with type II diabetes mellitus and chronic


renal failure on haemodialysis presents with
Test Analysis
unstable angina. She
currently uses twice daily insulin for glycaemic control but
control has been poor of late.

Investigations:

Haemoglobin 86 g/L (115-165)

MCV 84 fL (80-96)

Platelets 198 ×109/L (150-400)

White cell Count 6.7 ×109/L (4-11)

90 mmol/mol (20-46)
HbA1c
10.4% (3.8-6.4)

What is the most appropriate treatment?

(Please select 1 option)



Blood transfusion with two units of packed red cells Score:


Intravenous iron infusion Total Answered:


Oral ferrous sulphate


Subcutaneous erythropoietin
Question Navigator


Subcutaneous erythropoietin and intravenous iron
Tags

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Work Smart MRCP Part 2 Written 2003 December

A 58-year-old lady with type II diabetes mellitus and chronic


renal failure on haemodialysis presents with
Answer Statistics
unstable angina. She
currently uses twice daily insulin for glycaemic control but
control has been poor of late.

Investigations: Test Analysis


Haemoglobin 86 g/L (115-165)

MCV 84 fL (80-96)

Platelets 198 ×109/L (150-400)

White cell Count 6.7 ×109/L (4-11)

90 mmol/mol (20-46)
HbA1c
10.4% (3.8-6.4)

What is the most appropriate treatment?

(Please select 1 option)


Blood transfusion with two units of packed red cells
Correct


Intravenous iron infusion
Score:

Oral ferrous sulphate
Total Answered:

Subcutaneous erythropoietin


Subcutaneous erythropoietin and intravenous iron
Feedback

The aim in all patients with chronic renal failure is to


maintain the haemoglobin concentration above 11 g/dL (K- Question Navigator
DOQI
guidelines). It is important to correct anaemia, as it leads to
ventricular hypertrophy, which increases the
cardiovascular
morbidity and mortality. Revision Notes
This patient is exhibiting signs of severe anaemia, i.e. angina.
This may or may not be related to underlying
coronary artery
disease. The treatment of choice is an urgent blood transfusion, to
treat her ongoing symptoms. Tags
If this patient did not have symptoms the correct answer would
be subcutaneous erythropoietin and intravenous
iron.

IV iron therapy should be given (following a small IV test dose,


to exclude anaphylaxis) and SC erythropoietin, to
enhance
erythropoiesis and increase the haemoglobin concentration.

If the patient did not respond to this dual regime, she should
be investigated for sepsis, chronic blood loss, non-
compliance of
treatment, or severe hyperparathyroidism (which can cause marrow
fibrosis).

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Work Smart MRCP Part 2 Written July 2011

A 55-year-old man was referred to the outpatient clinic with


anaemia.
Test Analysis
He had presented to his general practitioner with a three month
history of fatigue and low back pain. There was
no preceding
history of trauma. A plain x ray of his lumbar spine showed a lytic
lesion in the body of the fourth
lumbar vertebra (L4).

Investigations revealed:

Haemoglobin 105 g/L (130-180)

WBC 4.0 ×109/L (4-11)

Platelets 175 ×109/L (150-400)

Serum sodium 137 mmol/L (137-144)

Serum potassium 3.5 mmol/L (3.5-4.9)

Serum urea 2.7 mmol/L (2.5-7.5)

Serum creatinine 110 µmol/L (60-110)


Score:
Serum corrected calcium 2.4 mmol/L (2.2-2.6)
Total Answered:
Bence Jones proteins were detected in the urine.

A skeletal survey showed increased uptake in the L4. Question Navigator


Which of the following therapies will reduce the risk of
pathological fracture?
Tags
(Please select 1 option)

Autologous stem cell transplantation


Melphalan


Pamidronate


Plasmapheresis


Steroids

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Work Smart MRCP Part 2 Written July 2011

A 55-year-old man was referred to the outpatient clinic with


anaemia.
Answer Statistics
He had presented to his general practitioner with a three month
history of fatigue and low back pain. There was
no preceding
history of trauma. A plain x ray of his lumbar spine showed a lytic
lesion in the body of the fourth Test Analysis
lumbar vertebra (L4).

Investigations revealed:

Haemoglobin 105 g/L (130-180)

WBC 4.0 ×109/L (4-11)

Platelets 175 ×109/L (150-400)

Serum sodium 137 mmol/L (137-144)

Serum potassium 3.5 mmol/L (3.5-4.9)

Serum urea 2.7 mmol/L (2.5-7.5)

Serum creatinine 110 µmol/L (60-110)

Serum corrected calcium 2.4 mmol/L (2.2-2.6)

Bence Jones proteins were detected in the urine. Score:

A skeletal survey showed increased uptake in the L4. Total Answered:

Which of the following therapies will reduce the risk of


pathological fracture?
Feedback
(Please select 1 option)
Autologous stem cell transplantation
Incorrect answer selected
Question Navigator

Melphalan

Pamidronate
This is the correct answer Revision Notes

Plasmapheresis
Tags

Steroids

Pathological fractures and bone pain are extremely common


symptoms in patients with myeloma.

It is estimated that 70% of patients have bone pain at the time


of presentation.

Biphosphonates reduce bony disease in myeloma, lowering the


frequency of pathological fractures. There is also
evidence that
bisphosphonates modulate the disease and have some antitumour
activity. As a result,
bisphosphonates should be given routinely to
patients with myeloma, even in the absence of hypercalcaemia.

Further Reading:
1. Kyle RA. The Role of Bisphosphonates in Multiple
Myeloma. Ann Int Med 2000; 132: 734-6
2. Shipman C, et al. Bisphosphonates induce apoptosis in human myeloma
cell lines: a novel antitumour
activity. Br J Haematol
1997; 98: 665-72

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A 45-year-old male presents with a one year history of impotence
and reduced shaving frequency.
Test Analysis
These symptoms have evolved over this period of time and he is
unaware of any erections whatsoever and has
no libido. He also is
aware that he shaves just once weekly whereas previously he was
shaving daily. Together
with these symptoms he has also been
feeling rather lethargic with reduced energy and is aware of vague
joint
aches.

He takes no medication and has otherwise been well except for an


appendicectomy at the age of 20. He drinks
approximately 20 units
of alcohol weekly and smokes five cigarettes daily. He is married
but has no children.

On examination, he appears slightly pigmented, has gynaecomastia


and a fine skin with scant facial, pubic and
axillary hair.
Examination of the testes reveal testicular size of approximately
15 mls bilaterally and no masses
are felt. Examination of the
cardiovascular, respiratory and abdominal systems are normal. No
abnormalities are
noted on joint movements with a full range of
movement.

Investigations reveal:

Prolactin 370 mU/L (50-450)


Score:
Testosterone 3.5 nmol/L (10-30)

LH 2.1 mU/L (2-10)


Total Answered:

FSH 2 mU/L (2-10)


Question Navigator
Free T4 12.8 pmol/L (10-22)

TSH 2.1 mU/L (0.4-5)


Tags
Which of the following is the most important investigation for
this patient?

(Please select 1 option)



Ferritin


Karyotype


MRI pituitary


Short Synacthen test


Ultrasound examination of testes

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Work Smart
A 45-year-old male presents with a one year history of impotence
and reduced shaving frequency.
Answer Statistics
These symptoms have evolved over this period of time and he is
unaware of any erections whatsoever and has
no libido. He also is
aware that he shaves just once weekly whereas previously he was
shaving daily. Together Test Analysis
with these symptoms he has also been
feeling rather lethargic with reduced energy and is aware of vague
joint
aches.

He takes no medication and has otherwise been well except for an


appendicectomy at the age of 20. He drinks
approximately 20 units
of alcohol weekly and smokes five cigarettes daily. He is married
but has no children.

On examination, he appears slightly pigmented, has gynaecomastia


and a fine skin with scant facial, pubic and
axillary hair.
Examination of the testes reveal testicular size of approximately
15 mls bilaterally and no masses
are felt. Examination of the
cardiovascular, respiratory and abdominal systems are normal. No
abnormalities are
noted on joint movements with a full range of
movement.

Investigations reveal:

Prolactin 370 mU/L (50-450)

Testosterone 3.5 nmol/L (10-30)

LH 2.1 mU/L (2-10)


Score:
FSH 2 mU/L (2-10)
Total Answered:
Free T4 12.8 pmol/L (10-22)

TSH 2.1 mU/L (0.4-5)


Feedback
Which of the following is the most important investigation for
this patient?

(Please select 1 option) Question Navigator


Ferritin
Correct
Revision Notes

Karyotype


MRI pituitary Tags

Short Synacthen test


Ultrasound examination of testes

This man has hypogonadotrophic hypogonadism (low testosterone


but luteinising hormone [LH] and follicle-
stimulating hormone [FSH]
are inappropriately low normal) indicating pituitary
involvement.

But the features of this disorder in a middle aged male with a


pigmented appearance, gynaecomastia and
arthralgia would suggest a
diagnosis of haemochromatosis and a ferritin would be the most
appropriate
investigation.

Testes appear normal and the issue is with the pituitary so


ultrasound is unnecessary.

Addison's is not suggested by the symptoms - no weight loss


mentioned, no postural features so a short
Synacthen test would not
be the best choice.

It could be argued that pituitary MRI is appropriate as a


pituitary tumour could produce these results. However
with a
macroadenoma and stalk compression, prolactin may be slightly
higher, visual fields may be abnormal
and the arthralgia with
gynaecomastia could not be explained.
Finally, LH and FSH would be high in Klinefelter's and the
history also suggests that previously erectile function
and
development were normal.

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Work Smart MRCP Part 2 Written 2006 December

A 33-year-old woman was referred by her GP with a four month


history of weight loss and agitation.
Test Analysis
On examination she had a fine tremor, a resting tachycardia and
a smooth goitre.

Investigations showed:

Free T4 32.1 pmol/L (10-22)

Free T3 10.2 pmol/L (5-10)

TSH <0.02 mU/L (0.4-5)

She was commenced on carbimazole 40 mg daily together with


propranolol 120 mg daily. One month later she
re-attended clinic
feeling better but gave a three day history of a sore throat. No
abnormalities were found on
examination of the throat and she was
apyrexial.

Investigations reveal:

Haemoglobin 115 g/L (115-165)

MCV 80 fL (80-96) Score:


Platelets 155 ×109/L (150-400) Total Answered:
White cell count 4.1 ×109/L (4-11)

Neutrophils 2.1 ×109/L (1.5-7)


Question Navigator

Lymphocytes 1.5 ×109/L (1.5-4)


Tags
What is the most appropriate management for this patient?

(Please select 1 option)



Admit to ward with barrier nursing


Reassure and continue carbimazole


Repeat blood tests in one week


Stop carbimazole


Treat with antibiotics

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Work Smart MRCP Part 2 Written 2006 December

A 33-year-old woman was referred by her GP with a four month


history of weight loss and agitation.
Answer Statistics
On examination she had a fine tremor, a resting tachycardia and
a smooth goitre.

Investigations showed: Test Analysis

Free T4 32.1 pmol/L (10-22)

Free T3 10.2 pmol/L (5-10)

TSH <0.02 mU/L (0.4-5)

She was commenced on carbimazole 40 mg daily together with


propranolol 120 mg daily. One month later she
re-attended clinic
feeling better but gave a three day history of a sore throat. No
abnormalities were found on
examination of the throat and she was
apyrexial.

Investigations reveal:

Haemoglobin 115 g/L (115-165)

MCV 80 fL (80-96)

Platelets 155 ×109/L (150-400)


Score:
White cell count 4.1 ×109/L (4-11)
Total Answered:
Neutrophils 2.1 ×109/L (1.5-7)

Lymphocytes 1.5 ×109/L (1.5-4)


Feedback
What is the most appropriate management for this patient?
Question Navigator
(Please select 1 option)
Admit to ward with barrier nursing
Incorrect answer selected
Revision Notes
Reassure and continue carbimazole
This is the correct answer


Repeat blood tests in one week Tags

Stop carbimazole


Treat with antibiotics

There are no abnormalities on this young woman's full blood


count (FBC) except for a probable iron deficiency
that probably
relates to menstruation.

Frequently lowish white blood cells (WBC) are noted in


thyrotoxicosis per se, as well as following the introduction
of
thionamides, and this is not an indication to stop therapy.
However, therapy should be stopped if there is a
demonstrable
neutropenia/agranulocytosis with neutrophil count below 1.5
×109/L (1.5-7).

Carbimazole-induced agranulocytosis (neutrophil


count below 0.5 ×109/L) is fortunately rare, but life
threatening.
Thionamides should be withdrawn, infection treated
with appropriate antibiotics (broad spectrum cephalosporin)
and
occasionally, granulocyte colony-stimulating factor (G-CSF) is
required when white count fails to respond.

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A 61-year-old old lady was referred to clinic by her GP with a
six month history of fatigue. There was no history of
Test Analysis
weight loss
or change in bowel habit, no cough or sputum production.

She denied having any fevers or sweats. Her only other complaint
was of occasional pains in her joints
secondary to rheumatoid
arthritis, which had been diagnosed three years previously.

She worked as an assistant in a bookshop and lived with her


husband. She had three children, all of whom were
well. There was
no family history of note. She was a lifelong non-smoker and seldom
drank alcohol.

On examination she appeared well. She was afebrile. Xanthelasma


were noted around both eyes. A few faint
scratch marks were visible
on the skin of her arms. She attributed this to an
'allergy' to a new washing powder.

Her pulse was 70/min with blood pressure 110/65 mmHg. Heart
sounds were normal with no murmurs or added
sounds. Her chest was
clear. The abdomen was soft and non-tender. The spleen was palpable
3 centimetres
below the left costal margin. The liver and kidneys
were not palpable. Neurological examination was normal.

What is the most likely diagnosis?

(Please select 1 option)


Score:

Chronic lymphocytic leukaemia (CLL)
Total Answered:

Familial hypercholesterolaemia


Hodgkin's lymphoma
Question Navigator

Primary biliary cirrhosis


Pseudoxanthoma elasticum Tags

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A 61-year-old old lady was referred to clinic by her GP with a
six month history of fatigue. There was no history of
Answer Statistics
weight loss
or change in bowel habit, no cough or sputum production.

She denied having any fevers or sweats. Her only other complaint
was of occasional pains in her joints Test Analysis
secondary to rheumatoid
arthritis, which had been diagnosed three years previously.

She worked as an assistant in a bookshop and lived with her


husband. She had three children, all of whom were
well. There was
no family history of note. She was a lifelong non-smoker and seldom
drank alcohol.

On examination she appeared well. She was afebrile. Xanthelasma


were noted around both eyes. A few faint
scratch marks were visible
on the skin of her arms. She attributed this to an
'allergy' to a new washing powder.

Her pulse was 70/min with blood pressure 110/65 mmHg. Heart
sounds were normal with no murmurs or added
sounds. Her chest was
clear. The abdomen was soft and non-tender. The spleen was palpable
3 centimetres
below the left costal margin. The liver and kidneys
were not palpable. Neurological examination was normal.

What is the most likely diagnosis?

(Please select 1 option)


Chronic lymphocytic leukaemia (CLL)
Incorrect answer selected


Familial hypercholesterolaemia Score:

Hodgkin's lymphoma Total Answered:
Primary biliary cirrhosis
This is the correct answer


Pseudoxanthoma elasticum Feedback

Question Navigator
Over 90% of cases of primary biliary cirrhosis (PBC) occur in
women aged 35 to 70.

The presentation is usually insidious. Pruritus, non-specific


fatigue, or both are the initial symptoms in more than Revision Notes
50% of
patients and can precede other symptoms by months or years.

About 50% of patients present with an enlarged, firm, non-tender


liver; 25%, with splenomegaly; about 15%, with Tags
skin xanthomas; and
10%, with hyperpigmentation.

Jaundice is present in 20% of patients and tends to develop in


others with time.

Clubbing, metabolic bone disease (that is, osteoporosis),


peripheral neuropathy, renal tubular acidosis, and
steatorrhea
(from the cholestasis and pancreatic secretory failure) are
possible. Later, all the features and
complications of cirrhosis
can develop.

PBC is commonly associated with autoimmune disorders (for


example, RA, scleroderma, sicca complex,
autoimmune
thyroiditis).

CLL is usually associated with symmetrical lymphadenopathy;


splenomegaly is detectable in about 50% of
cases.

Localised lymphadenopathy (usually above the diaphragm) is the


commonest presentation of Hodgkin's disease;
hepatosplenomegaly
is usually associated with NHL.

While familial hypercholesterolaemia might explain the


xanthelasma in this case, it does not adequately explain
the other
symptoms of fatigue and pruritus or the presence of
splenomegaly.
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Work Smart MRCP Part 2 Written 2004 August

A 32-year-old Nigerian lady with sickle cell anaemia (Hb SS) has
a history of recurrent back pain.
Test Analysis
She presents to casualty with fever and a worsening of the back
pain. There is no history of weight loss or night
sweats.

Investigations show:

Haemoglobin 78 g/L (115-165)

White cell count 10.1 ×109/L (4-11)

Platelets 475 ×109/L (150-400)

Reticulocytes 12%

Serum total bilirubin 88 µmol/L (1-22)

What is the most likely diagnosis?

(Please select 1 option)



Aplastic crisis Score:


Haemolytic crisis Total Answered:


Malaria
Question Navigator

Tuberculosis


Vasoocclusive event
Tags

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Work Smart MRCP Part 2 Written 2004 August

A 32-year-old Nigerian lady with sickle cell anaemia (Hb SS) has
a history of recurrent back pain.
Answer Statistics
She presents to casualty with fever and a worsening of the back
pain. There is no history of weight loss or night
sweats. Test Analysis
Investigations show:

Haemoglobin 78 g/L (115-165)

White cell count 10.1 ×109/L (4-11)

Platelets 475 ×109/L (150-400)

Reticulocytes 12%

Serum total bilirubin 88 µmol/L (1-22)

What is the most likely diagnosis?

(Please select 1 option)


Aplastic crisis
Incorrect answer selected


Haemolytic crisis
Score:

Malaria
Total Answered:

Tuberculosis

Vasoocclusive event
This is the correct answer
Feedback

The blood results are suggestive of a sickling episode. Question Navigator

The Hb is low with a raised reticulocyte count and


hyperbilirubinaemia. There is no evidence of an aplastic crisis
Revision Notes
as
the Hb is reasonable (remember, patients with sickle cell tend to
run with a Hb between 70-90 g/L normally),
with a good reticulocyte
count.
Tags
The anemia of SC is usually a chronic, reasonably
well-compensated hemolytic anemia with an appropriate

reticulocytosis. For example, the mean hemoglobin and hematocrit


concentrations on average may be 79 g/L
and 22.9% respectively,
with a reticulocyte count of between 3-15%.

Due to decreased life span of the erythrocyte, average 17 days


(normal 120 days), there is also a chronic
circulating unconjugated
hyperbilirubinaemia.

Conversely, the Hb not low enough and reticulocyte count and


bilirubin not high enough for a haemolytic crisis.

Vasoocclusive events can cause bone pain. Triggers include


infection, dehydration , alcohol and change in
temperature.

Likely infection is the cause in this case as patient has


fever.

TB and malaria are not as likely from the clinical scenario.

Homozygotes for HbS can get malaria.

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Work Smart MRCP Part 2 Written 2004 August

A 26-year-old man attends the dental hospital for a tooth


extraction.
Test Analysis
He reports prolonged bleeding after a tooth extraction two years
previously. He has had no other surgical
challenges and no other
history of prolonged bleeding. He is otherwise fit and well.

On further questioning he said his mother had recently been


referred to a local hospital for recurrent nose bleeds
and
menorrhagia. He was also aware that his sister had been seen in the
past for menorrhagia.

The dental extraction cannot be delayed.

What should the dentist be advised to give the patient to reduce


the risk of significant bleeding?

(Please select 1 option)



Cryoprecipitate


Factor VIII replacement


Fresh frozen plasma (FFP)


Tranexamic acid
Score:

von Willebrand factor concentrate
Total Answered:

Question Navigator
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Work Smart MRCP Part 2 Written 2004 August

A 26-year-old man attends the dental hospital for a tooth


extraction.
Answer Statistics
He reports prolonged bleeding after a tooth extraction two years
previously. He has had no other surgical
challenges and no other
history of prolonged bleeding. He is otherwise fit and well. Test Analysis
On further questioning he said his mother had recently been
referred to a local hospital for recurrent nose bleeds
and
menorrhagia. He was also aware that his sister had been seen in the
past for menorrhagia.

The dental extraction cannot be delayed.

What should the dentist be advised to give the patient to reduce


the risk of significant bleeding?

(Please select 1 option)


Cryoprecipitate
Incorrect answer selected


Factor VIII replacement


Fresh frozen plasma (FFP)

Tranexamic acid
This is the correct answer


von Willebrand factor concentrate

Score:

The bleeding described is a defect of primary haemostasis. It is


inherited as mother and sister are affected. Total Answered:

The most likely diagnosis is von Willebrand (vWB) disease. It is


likely to be mild (type I) as the patient and family
members have
reached a good age with no major bleeding problems. Feedback
The most appropriate management is therefore good surgical
technique, pressure and oral tranexamic acid. The
side effects are
minimal. Question Navigator

The choice of treatment for a mild vWB facing a more invasive


procedure would be DDAVP, providing there is no
Revision Notes
contraindication.
vWB factor concentrate would be reserved as second line treatment
to DDAVP.

FFP and cryoprecipitate are not used in von Willebrand


disease. Tags
Haemophilia is not the diagnosis with a defect of primary
haemostasis and generally females are not affected as
it is X
linked.

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Work Smart MRCP Part 2 Written 2004 August

An inpatient on the haematology ward develops a fever five


minutes after starting a platelet transfusion. His
Test Analysis
temperature
rises 1ºC from pre-transfusion observations.

Other than feeling hot he is well. His blood pressure, pulse and
oxygen saturations are all unchanged from pre-
transfusion
observations. An hour later he remains febrile, but is well and all
other observations stable.

What is the most likely cause of his fever?

(Please select 1 option)



ABO incompatibility


Anaphylactoid reaction


Bacterial contamination of transfused platelets


Leucocyte cytokines present in unit transfused


Platelet destruction due to IgG

Score:

Total Answered:
Submit answer
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Work Smart MRCP Part 2 Written 2004 August

An inpatient on the haematology ward develops a fever five


minutes after starting a platelet transfusion. His
Answer Statistics
temperature
rises 1ºC from pre-transfusion observations.

Other than feeling hot he is well. His blood pressure, pulse and
oxygen saturations are all unchanged from pre- Test Analysis
transfusion
observations. An hour later he remains febrile, but is well and all
other observations stable.

What is the most likely cause of his fever?

(Please select 1 option)


ABO incompatibility
Incorrect answer selected


Anaphylactoid reaction


Bacterial contamination of transfused platelets

Leucocyte cytokines present in unit transfused


This is the correct answer


Platelet destruction due to IgG

With such a rapid rise in temperature one needs to consider a


haemolytic transfusion reaction due to ABO
incompatibility.
However, several factors weigh against this as the diagnosis.
Score:
He is receiving a platelet transfusion, which does not typically
contain the antigens necessary to provoke a
Total Answered:
transfusion reaction.
It is possible that the plasma in which the platelets are suspended
contains a high titre of
anti-A and anti-B antibodies, but this
would be unusual. In addition, the clinical picture is not entirely
consistent
with ABO incompatibility: apart from a mild fever, the
patient is very well. With ABO incompatibility patients Feedback
become
shocked very quickly.

Febrile non-haemolytic reactions are very common, and are due to


the presence of pyrogenic cytokines released
Question Navigator
from leucocytes during
storage of the platelet units. This is the most likely cause in
this patient's case. The
incidence is decreasing due to
leucodepletion of blood products. Revision Notes
Bacterial contamination of platelets is common, due to the fact
that they are stored at 22ºC. This supports
growth; but the
temperature rise on transfusion is not usually as rapid. Tags

Destruction of platelets by IgG is the process behind immune


thrombocytopenia. Platelets are not usually
transfused in this
condition.

The history does not sound like an anaphylactoid reaction.

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A 56-year-old man with chronic renal failure attended the renal
clinic.
Test Analysis
He was undergoing regular haemodialysis and had been treated for
the past six months with oral ferrous
sulphate (200 mg three times
a day). His haemoglobin in clinic measured at 76 g/L, compared with
106 g/L six
months previously.

Which of the following is the most appropriate treatment?

(Please select 1 option)



Erythropoietin


Increase the dose of oral ferrous sulphate


Intravenous iron


Intravenous iron and subcutaneous erythropoietin


Transfusion of two units of packed red cells

Score:

Total Answered:
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Work Smart
A 56-year-old man with chronic renal failure attended the renal
clinic.
Answer Statistics
He was undergoing regular haemodialysis and had been treated for
the past six months with oral ferrous
sulphate (200 mg three times
a day). His haemoglobin in clinic measured at 76 g/L, compared with
106 g/L six Test Analysis
months previously.

Which of the following is the most appropriate treatment?

(Please select 1 option)


Erythropoietin
Incorrect answer selected


Increase the dose of oral ferrous sulphate


Intravenous iron

Intravenous iron and subcutaneous erythropoietin


This is the correct answer


Transfusion of two units of packed red cells

The aim in this patient is to maintain the haemoglobin


concentration above 110 g/L (K-DOQI guidelines). It is
important to
correct anaemia, as it leads to ventricular hypertrophy, which
increases the cardiovascular
Score:
morbidity and mortality.
Total Answered:
This patient's haemoglobin has dropped despite appropriate
doses of oral ferrous sulphate. The iron demands
will increase
further if erythropoietin is started.
Feedback
The patient should be prescribed IV iron therapy (following a
small IV test dose, to exclude anaphylaxis) and SC
erythropoietin,
to enhance erythropoiesis and increase the haemoglobin
concentration.
Question Navigator
If the patient does not respond to this dual regime, he should
be investigated for sepsis, chronic blood loss, non-
compliance of
treatment, or severe hyperparathyroidism (which can cause marrow
fibrosis).
Revision Notes

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Work Smart MRCP Part 2 Written 2004 August

A 64-year-old lady is reviewed in the outpatient clinic. She has


been known to have chronic lymphocytic
Test Analysis
leukaemia (CLL) for six
months.

Apart from three chest infections in the last year, she is


otherwise well.

Investigations show:

Haemoglobin 134 g/L (115-165)

White cell count 30.2 ×109/L (4-11)

Lymphocytes 26.2 ×109/L (1.5-4)

Neutrophils 3.8 ×109/L (1.5-7)

Platelet count 350 ×109/L (150-400)

Serum electrophoresis:

IgG 2.5 g/L (6-13)


Score:
IgA 0.2 g/L (0.8-3.0)
Total Answered:
IgM 0.1 g/L (0.4-2.5)

What is the most appropriate management option at this time? Question Navigator
(Please select 1 option)

Chlorambucil
Tags


Fludarabine


Intravenous immunoglobulin infusions


Observe


Stem cell transplant

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Work Smart MRCP Part 2 Written 2004 August

A 64-year-old lady is reviewed in the outpatient clinic. She has


been known to have chronic lymphocytic
Answer Statistics
leukaemia (CLL) for six
months.

Apart from three chest infections in the last year, she is


otherwise well. Test Analysis
Investigations show:

Haemoglobin 134 g/L (115-165)

White cell count 30.2 ×109/L (4-11)

Lymphocytes 26.2 ×109/L (1.5-4)

Neutrophils 3.8 ×109/L (1.5-7)

Platelet count 350 ×109/L (150-400)

Serum electrophoresis:

IgG 2.5 g/L (6-13)

IgA 0.2 g/L (0.8-3.0)

IgM 0.1 g/L (0.4-2.5) Score:

What is the most appropriate management option at this time? Total Answered:

(Please select 1 option)


Chlorambucil
Incorrect answer selected
Feedback


Fludarabine
Question Navigator
Intravenous immunoglobulin infusions
This is the correct answer


Observe Revision Notes

Stem cell transplant
Tags

CLL is considered a low grade lymphoproliferative disorder.

An observation policy is usual during the early stages of the


disease. If disease control is necessary for B
symptoms or a
rapidly rising white cell count or bone marrow compromise (anaemia
and thrombocytopenia),
chlorambucil would be the choice.

Recurrent infections are recognised in CLL due to


hypogammaglobulinaemia and immune paresis; but are not an

indication for disease control.

She is likely to benefit from regular infusions of


immunoglobulin to prevent infections.

In a patient of this age fludarabine might be used as second


line treatment in CLL.

Stem cell transplant is not initial treatment for CLL.

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Work Smart MRCP Part 2 Written 2004 August

A 15-year-old girl is referred urgently to clinic because of a


two day history of spontaneous bruising.
Test Analysis
She has no past history of note and is not taking any regular
prescribed medication. She has noticed
spontaneous appearance of
bruises on her hips, thighs and upper arms over the past three
days. There is no
history of trauma to account for their
appearance. The largest of these measures 15 cm in diameter.
Otherwise
she feels well, though she reports having had a mild
viral illness two weeks previously.

Investigations show:

Haemoglobin 141 g/L (115-165)

White blood cells 7.3 ×109/L (4-11)

Platelets 15 ×109/L (150-400)

What is the most important next step?

(Please select 1 option)



Blood film examination
Score:

Bone marrow biopsy
Total Answered:

Check coagulation screen.


Reassure that this is likely to resolve and see again in five days Question Navigator

Start prednisolone treatment
Tags

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Work Smart MRCP Part 2 Written 2004 August

A 15-year-old girl is referred urgently to clinic because of a


two day history of spontaneous bruising.
Answer Statistics
She has no past history of note and is not taking any regular
prescribed medication. She has noticed
spontaneous appearance of
bruises on her hips, thighs and upper arms over the past three
days. There is no Test Analysis
history of trauma to account for their
appearance. The largest of these measures 15 cm in diameter.
Otherwise
she feels well, though she reports having had a mild
viral illness two weeks previously.

Investigations show:

Haemoglobin 141 g/L (115-165)

White blood cells 7.3 ×109/L (4-11)

Platelets 15 ×109/L (150-400)

What is the most important next step?

(Please select 1 option)


Blood film examination
Correct


Bone marrow biopsy


Check coagulation screen. Score:


Reassure that this is likely to resolve and see again in five days Total Answered:


Start prednisolone treatment
Feedback

The likely diagnosis is immune thrombocytopenia. Question Navigator


The clues to this are that the girl is well. There is post viral
illness with quite marked thrombocytopenia but other
full blood
count (FBC) parameters are normal. Revision Notes
The diagnosis is one of exclusion, and in the absence of major
bleeding, management would be observation, as
it can resolve
spontaneously. Tags
The most important investigation is a blood film. Although not
diagnostic, this will confirm the FBC findings and
also exclude
more sinister pathology such as leukaemia.

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Work Smart MRCP Part 2 Written 2004 August

A 34-year-old man presents with a swollen, painful right


calf.
Test Analysis
A Doppler ultrasound confirms the presence of a posterior knee
occlusive deep vein thrombosis (DVT). Two
weeks previously a below
knee plaster cast had been removed on the right side. This had been
in place for four
weeks following a rugby injury. He had no other
significant past medical history.

What is the best management plan for this man?

(Please select 1 option)



Low molecular weight heparin followed by warfarin for six months


Low molecular weight heparin followed by warfarin for three months.


No treatment necessary


TED support stockings and repeat Doppler in one week.


Warfarin only

Score:

Total Answered:
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Work Smart MRCP Part 2 Written 2004 August

A 34-year-old man presents with a swollen, painful right


calf.
Answer Statistics
A Doppler ultrasound confirms the presence of a posterior knee
occlusive deep vein thrombosis (DVT). Two
weeks previously a below
knee plaster cast had been removed on the right side. This had been
in place for four Test Analysis
weeks following a rugby injury. He had no other
significant past medical history.

What is the best management plan for this man?

(Please select 1 option)


Low molecular weight heparin followed by warfarin for six months
Incorrect answer selected

Low molecular weight heparin followed by warfarin for three months.


This is the correct answer


No treatment necessary


TED support stockings and repeat Doppler in one week.


Warfarin only

This gentleman has a DVT secondary to an acquired risk factor


that has now been removed. Although the risk
factor is removed, the
patient should be anticoagulated.
Score:
Candidates should know that in this situation the duration of
treatment is three months.
Total Answered:
They should also know that warfarin should never be given alone,
without heparin, when treating a thrombotic
event. This is because
when warfarin is started, there is a pro-thrombotic state created,
by falling protein C and
Feedback
protein S (these are vitamin K dependent
natural anticoagulants) and these fall before factors II, VII, IX
and X.

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Work Smart MRCP Part 2 Written 2004 August

An 18-year-old Zimbabwean man presented with a two week history


of massive painless cervical
Test Analysis
lymphadenopathy.

Which of the following investigations should be performed to


obtain the diagnosis?

(Please select 1 option)



Bone marrow aspiration and trephine biopsy


Chest x ray


CT scan chest


HIV antibody test


Lymph node biopsy

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Score:

Total Answered:

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Work Smart MRCP Part 2 Written 2004 August

An 18-year-old Zimbabwean man presented with a two week history


of massive painless cervical
Answer Statistics
lymphadenopathy.

Which of the following investigations should be performed to


obtain the diagnosis? Test Analysis
(Please select 1 option)
Bone marrow aspiration and trephine biopsy
Incorrect answer selected


Chest x ray


CT scan chest


HIV antibody test

Lymph node biopsy


This is the correct answer

Given that the lymphadenopathy is massive and painless, the most


likely diagnosis is a lymphoproliferative
disorder or Hodgkin's
disease, rather than infection or other cause.

The mass has appeared rapidly and this would make you think of a
lymphoproliferative disorder with a short
doubling time, for
example, Burkitt's lymphoma. The clue to this is that he is
from Africa, with Burkitt's being
Score:
endemic in parts of Africa,
and can also be seen with immunodeficiency states such as HIV.
Total Answered:
Only a lymph node biopsy will give you a definitive
diagnosis.

A bone marrow and full body CT is done as part of staging a


lymphoproliferative disorder, but will not necessarily
Feedback
provide you
with a definitive diagnosis.

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Work Smart MRCP Part 2 Written 2004 August

A 75-year-old man is referred to the outpatient clinic for


investigation of anaemia. He gives a six month history of
Test Analysis
fatigue
and exertional dyspnoea. He has a past history of hypertension
which is controlled with atenolol, and had
a prostatectomy aged 70
years for benign prostatic hypertrophy. He smoked 20 cigarettes per
day until aged 38
years. He drinks approximately eight units of
alcohol per week.

On examination he appears pale. He becomes mildly dyspnoeic


getting onto the examination couch. His pulse is
100 beats per
minute, regular with blood pressure 125/65. His heart sounds are
normal with no murmurs and his
chest is clear. His abdomen is soft.
A smooth, non-tender liver edge is palpable 3 cm below the right
costal
margin; the spleen is palpable 10 cm below the left costal
margin.

His peripheral blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags

What is the most likely diagnosis?

(Please select 1 option)



Chronic lymphocytic leukaemia


Haemolytic anaemia


Multiple myeloma


Myelofibrosis


Visceral leishmaniasis

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Work Smart MRCP Part 2 Written 2004 August

A 75-year-old man is referred to the outpatient clinic for


investigation of anaemia. He gives a six month history of
Answer Statistics
fatigue
and exertional dyspnoea. He has a past history of hypertension
which is controlled with atenolol, and had
a prostatectomy aged 70
years for benign prostatic hypertrophy. He smoked 20 cigarettes per
day until aged 38
years. He drinks approximately eight units of
alcohol per week. Test Analysis

On examination he appears pale. He becomes mildly dyspnoeic


getting onto the examination couch. His pulse is
100 beats per
minute, regular with blood pressure 125/65. His heart sounds are
normal with no murmurs and his
chest is clear. His abdomen is soft.
A smooth, non-tender liver edge is palpable 3 cm below the right
costal
margin; the spleen is palpable 10 cm below the left costal
margin.

His peripheral blood film is shown below:

Score:

Total Answered:

Feedback

Question Navigator
What is the most likely diagnosis?
Revision Notes
(Please select 1 option)
Chronic lymphocytic leukaemia
Incorrect answer selected
Tags

Haemolytic anaemia


Multiple myeloma

Myelofibrosis
This is the correct answer


Visceral leishmaniasis

The patient is anaemic with massive splenomegaly and a lesser


degree of hepatomegaly. The blood film shows
numerous tear-drop
cells. One cell (lower left) shows basophilic stippling.

Myelofibrosis is a disease of the over 50s. As the bone marrow


fibroses, myelopoiesis switches to the liver and
spleen. Patients
are anaemic and thrombocytopaenic with abnormal clotting.
Splenomegaly may be massive;
splenectomy may be required.

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Work Smart MRCP Part 2 Written 2004 August

A 21-year-old medical student presents with a ten day history of


fevers and malaise. There is no history of note.
Test Analysis
On examination, he has enlarged lymph nodes palpable in both
supraclavicular fossae. The nodes are smooth,
mobile and
non-tender. There is no recent history of overseas travel. He
returned from his elective period in
Gambia three months
previously.

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags
What is the likely diagnosis?

(Please select 1 option)



Dracunculiasis


Loa loa infection


Onchocerciasis


Schistosomiasis


West African trypanosomiasis

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Work Smart MRCP Part 2 Written 2004 August

A 21-year-old medical student presents with a ten day history of


fevers and malaise. There is no history of note.
Answer Statistics
On examination, he has enlarged lymph nodes palpable in both
supraclavicular fossae. The nodes are smooth,
mobile and
non-tender. There is no recent history of overseas travel. He
returned from his elective period in Test Analysis
Gambia three months
previously.

His blood film is shown below:

Score:

Total Answered:

Feedback
What is the likely diagnosis?

  Question Navigator

(Please select 1 option)


Revision Notes
Dracunculiasis
Incorrect answer selected


Loa loa infection Tags

Onchocerciasis


Schistosomiasis

West African trypanosomiasis


This is the correct answer

The blood film shows typical trypomastigotes of African


trypanosomiasis.

The causative agent is Trypanosoma brucei and is


transmitted to humans by the bite of the tsetse fly.

African trypanosomiasis has two recognised forms


East African sleeping sickness is caused by Trypanosoma
brucei rhodesiense
West African sleeping sickness is caused by Trypanosma
brucei gambiense.

The two forms of the disease are not distinguishable from their
macroscopic appearance. However, their clinical
manifestations are
different.

West African trypanosomiasis has a slower course. Symptoms start


several weeks or even months after the
tsetse fly bite.
The first stage of disease is haematolymphatic spread and is
accompanied by fever, and lymphadenopathy
(discrete, rubbery,
non-tender nodes). A rash sometimes occurs and mild
hepatosplenomegaly may develop.

The second stage is the meningoencephalitic stage. This occurs


months or years after the acquisition of
infection. Manifestations
include personality change and progressive indifference with
daytime somnolence.
Extrapyramidal signs and ataxia are common.

East African trypanosomiasis has similar clinical features, but


the progression of the disease is more rapid -
starting within days
of infection. Death may occur within weeks or months.

Rash is a more prominent feature and lymphadenopathy is less


frequently present.

Treatment is with suramin.

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Work Smart MRCP Part 2 Written 2004 August

A 29-year-old woman was admitted to hospital after having a


tonic-clonic seizure at home.
Test Analysis
She had been unwell for two days, firstly developing diarrhoea
and then complaining of fever and malaise. Over
the preceding 24
hours she had become slightly confused.

Investigations showed:

Haemoglobin 92 g/L (115-165)

White cell count 9.4 ×109/L (4-11)

Platelets 33 ×109/L (150-400)

Serum sodium 135 mmol/L (137-144)

Serum potassium 5.1 mmol/L (3.5-4.9)

Serum urea 34 mmol/L (2.5-7.5)

Serum creatinine 412 µmol/L (60-110)

PT 12 s (11.5-15.5) Score:
APTT 38 s (30-40)
Total Answered:
Fibrinogen 2.5 g/L (1.8-5.4)

Fibrin degradation products 85 mg/L (<100)


Question Navigator
Urine microscopy:
Tags
Blood +++

Protein +++

Her blood film is shown below:

Infection with which of the following organisms is most likely


to be the cause of her illness?

(Please select 1 option)



Escherichia coli

Herpes simplex virus-1


Neisseria meningitidis


Salmonella typhi


Streptococcus pneumoniae

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Work Smart MRCP Part 2 Written 2004 August

A 29-year-old woman was admitted to hospital after having a


tonic-clonic seizure at home.
Answer Statistics
She had been unwell for two days, firstly developing diarrhoea
and then complaining of fever and malaise. Over
the preceding 24
hours she had become slightly confused. Test Analysis
Investigations showed:

Haemoglobin 92 g/L (115-165)

White cell count 9.4 ×109/L (4-11)

Platelets 33 ×109/L (150-400)

Serum sodium 135 mmol/L (137-144)

Serum potassium 5.1 mmol/L (3.5-4.9)

Serum urea 34 mmol/L (2.5-7.5)

Serum creatinine 412 µmol/L (60-110)

PT 12 s (11.5-15.5)

APTT 38 s (30-40)

Fibrinogen 2.5 g/L (1.8-5.4) Score:


Fibrin degradation products 85 mg/L (<100) Total Answered:

Urine microscopy:
Feedback
Blood +++

Protein +++
Question Navigator
Her blood film is shown below:
Revision Notes

Tags

Infection with which of the following organisms is most likely


to be the cause of her illness?

(Please select 1 option)


Escherichia coli
Correct

Herpes simplex virus-1


Neisseria meningitidis


Salmonella typhi


Streptococcus pneumoniae

The blood film shows a microangiopathic haemolytic anaemia


(MAHA).

The combination of uraemia, anaemia, thrombocytopenia and MAHA


would suggest haemolytic uraemic
syndrome due to sepsis with
Escherichia coli 0157. The absence of any abnormality of
coagulation is typical.

Features of MAHA include red cell fragmentation and different


red cell sizes (anisocytosis).

Immature erythrocytes are released by the bone marrow in an


attempt to correct the anaemia. A nucleated red
blood cell is seen
near the centre of the slide; a neutrophil is shown on the
left.

Causes of MAHA include:


Thrombotic thrombocytopenic purpura (TTP)
Haemolytic uraemic syndrome
Disseminated intravascular coagulation
Haemolysis by prosthetic heart valves
Malignant hypertension
Septicaemia
Vasculitis
Connective tissue disease (polyarteritis nodosa [PAN],
Wegener's, systemic lupus erythematosus [SLE]).

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Work Smart MRCP Part 2 Written 2004 August

An 82-year old man presents to his general practitioner with a


six month history of fatigue and increasing
Test Analysis
exertional
dyspnoea.

Investigations show:

Haemoglobin 75 g/L (130-180)

White blood cells 3.12 ×109/L (4-11)

Neutrophils 34% -

Blasts 1% -

Platelets 12 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags

A bone marrow aspirate, stained with an iron stain shows:


What is the most likely diagnosis to explain his symptoms?

(Please select 1 option)



Bone marrow aplasia


Chronic myeloid leukaemia


Metastatic bone marrow infiltration


Myelodysplastic syndrome


Myelofibrosis

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Work Smart MRCP Part 2 Written 2004 August

An 82-year old man presents to his general practitioner with a


six month history of fatigue and increasing
Answer Statistics
exertional
dyspnoea.

Investigations show: Test Analysis


Haemoglobin 75 g/L (130-180)

White blood cells 3.12 ×109/L (4-11)

Neutrophils 34% -

Blasts 1% -

Platelets 12 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Feedback

Question Navigator

Revision Notes

Tags
A bone marrow aspirate, stained with an iron stain shows:
What is the most likely diagnosis to explain his symptoms?

(Please select 1 option)


Bone marrow aplasia
Incorrect answer selected


Chronic myeloid leukaemia


Metastatic bone marrow infiltration

Myelodysplastic syndrome
This is the correct answer


Myelofibrosis

The patient has a macrocytic anaemia, thrombocytopenia and


neutropenia with a small number of circulating
blasts (as shown on
the blood film). This suggests a diagnosis of myelodysplastic
syndrome (MDS).

Further evidence in support of a diagnosis of MDS comes from the


blood film, showing a dimorphic picture (some
red cells are
hypochromic and microcytic, while others appear macrocytic), and
the neutrophils are hypogranular
and hyposegmented (Pelger-Huet
cells).

The bone marrow shows ring sideroblasts with an iron stain. Ring
sideroblasts contain an abnormally high
concentration of iron -
usually stored in perinuclear mitochondria. Perl's stain (which
stains for iron) shows this
iron deposition as a dark ring around
the margin of the nucleus.

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Work Smart MRCP Part 2 Written 2004 August

A 50-year-old man presented with lethargy and weight loss.


Test Analysis
Investigations showed:

Hb 94 g/L (130-180)

WBC 54.2 ×109/L (4-11)

Platelets 79 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags

What would be your initial management plan for this patient?

(Please select 1 option)



Allogeneic bone marrow transplant


Alpha Interferon


AML type chemotherapy approach


Glivec (Imatinib)


Observation

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Work Smart MRCP Part 2 Written 2004 August

A 50-year-old man presented with lethargy and weight loss.


Answer Statistics
Investigations showed:

Hb 94 g/L (130-180)
Test Analysis

WBC 54.2 ×109/L (4-11)

Platelets 79 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Feedback

Question Navigator

What would be your initial management plan for this patient?


Revision Notes
(Please select 1 option)
Allogeneic bone marrow transplant
Incorrect answer selected Tags

Alpha Interferon


AML type chemotherapy approach

Glivec (Imatinib)
This is the correct answer


Observation

The blood film shows chronic myeloid leukaemia. Unlike CLL, CML
will usually progress to frank leukaemia quite
rapidly, so
treatment is needed. You should undertake a period of observation,
unless age and other co-
morbidity make treatment inappropriate.

NICE have now approved the tyrosine kinase inhibitor Glivec as


first line treatment for chronic and accelerated
phase CML.
Interferon is no longer used as first line, unless in the context
of a clinical trial. If the patient had
been in blast crisis phase,
then AML type chemotherapy as well as Glivec would be the
choice.

If remission is not achieved with Glivec, then in a patient


under 60-65 years, an allogeneic transplant would be
considered if
there was a matched sibling donor; in a 50-year-old patient or
younger a matched unrelated donor
transplant would be considered
too.
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Work Smart MRCP Part 2 Written 2004 August

A 19-year-old man of Afro-Caribbean origin presents to the


Emergency department with frank haematuria.
Test Analysis
On further questioning he gives a history of polyuria and
nocturia over several months.

Investigations show:

Hb 67 g/L (130-180)

MCV 58.0 fL (80-96)

WBC 13.1 ×109/L (4-11)

Neurophils 10.1 ×109/L (1.5-7.0)

Platelets 503 ×109/L (150-400)

The blood film shows:

Score:

Total Answered:

Question Navigator

Tags

What is the likely cause of the urinary symptoms?

(Please select 1 option)



Diabetes mellitus


Glomerulonephritis


Renal calculi


Renal papillary necrosis


Urinary tract infection

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Work Smart MRCP Part 2 Written 2004 August

A 19-year-old man of Afro-Caribbean origin presents to the


Emergency department with frank haematuria.
Answer Statistics
On further questioning he gives a history of polyuria and
nocturia over several months.

Investigations show: Test Analysis

Hb 67 g/L (130-180)

MCV 58.0 fL (80-96)

WBC 13.1 ×109/L (4-11)

Neurophils 10.1 ×109/L (1.5-7.0)

Platelets 503 ×109/L (150-400)

The blood film shows:

Score:

Total Answered:

Feedback

Question Navigator

Revision Notes

Tags
What is the likely cause of the urinary symptoms?

(Please select 1 option)


Diabetes mellitus
Incorrect answer selected


Glomerulonephritis


Renal calculi

Renal papillary necrosis


This is the correct answer


Urinary tract infection

The patient has sickle cell disease.

There is a hypochromic microcytic anaemia with crescent shaped


cells on the film; one erythrocyte contains a
Howell-Jolly body -
an indicator of hyposplenism.

The hyposplenism is secondary to splenic infarcts.


Renal papillary necrosis is a well recognised chronic
complication of HbSS disease, and the history, full blood
count and
blood film all support this.

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An 18-year-old woman was referred to hospital with a six month
history of increasing fatigue. She had no other
Test Analysis
symptoms. Her only
medication was ferrous sulphate 100 mg once daily which she had
bought from the
pharmacy.

On examination she was pale. Her pulse was 90 beats per minute
and regular. Her blood pressure was 110/60
mmHg.

Investigations revealed:

Haemoglobin 65 g/L (115-165)

Haematocrit 0.19 (0.36-0.47)

MCV 118 fL (80-96)

MCH 33.0 pg (28-32)

White cell count 8.4 ×109/L (4-11)

Platelets 95 ×109/L (150-400)


Score:
Anti-parietal cell antibody Positive 1:1200
Total Answered:
What is the next most appropriate step in management?

(Please select 1 option)


Question Navigator

Blood transfusion


Bone marrow aspiration Tags

Give intramuscular vitamin B12 and oral folic acid


Give intravenous iron infusion


Increase dose of oral ferrous sulphate to 200 mg three times daily

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An 18-year-old woman was referred to hospital with a six month
history of increasing fatigue. She had no other
Answer Statistics
symptoms. Her only
medication was ferrous sulphate 100 mg once daily which she had
bought from the
pharmacy.
Test Analysis
On examination she was pale. Her pulse was 90 beats per minute
and regular. Her blood pressure was 110/60
mmHg.

Investigations revealed:

Haemoglobin 65 g/L (115-165)

Haematocrit 0.19 (0.36-0.47)

MCV 118 fL (80-96)

MCH 33.0 pg (28-32)

White cell count 8.4 ×109/L (4-11)

Platelets 95 ×109/L (150-400)

Anti-parietal cell antibody Positive 1:1200

What is the next most appropriate step in management? Score:


(Please select 1 option) Total Answered:
Blood transfusion
Incorrect answer selected


Bone marrow aspiration Feedback
Give intramuscular vitamin B12 and oral folic acid
This is the correct answer
Question Navigator

Give intravenous iron infusion


Increase dose of oral ferrous sulphate to 200 mg three times daily Revision Notes

Tags
The patient has pernicious anaemia. Although she is profoundly
anaemic she is not haemodynamically
compromised and therefore does
not require a blood transfusion.

If not already done, blood should be taken for B12


and folate levels. She should be started on an intensive
treatment
regime with intramuscular vitamin B12 and oral folic
acid. She will need to continue on treatment with
ferrous sulphate
as her iron stores are likely to be depleted rapidly once her
marrow starts functioning.

Giving oral folic acid without vitamin B12 would be


hazardous and could precipitate subacute combined
degeneration of
the spinal cord.

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This 16-year-old boy has a long history of recurrent infections
since childhood.
Test Analysis
 

Score:

Total Answered:

Question Navigator

Click and drag to move the ophthalmoscope. Tags


What is the most likely diagnosis?

(Please select 1 option)



Abetalipoproteinaemia


Chediak-Higashi syndrome


Chronic granulomatous disease


Kearns-Sayre syndrome


Type 1 diabetes mellitus

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This 16-year-old boy has a long history of recurrent infections
since childhood.
Answer Statistics
 

Test Analysis

Score:

Total Answered:

Click and drag to move the ophthalmoscope. Feedback


What is the most likely diagnosis?
Question Navigator
(Please select 1 option)
Abetalipoproteinaemia
Incorrect answer selected
Revision Notes
Chediak-Higashi syndrome
This is the correct answer


Chronic granulomatous disease Tags


Kearns-Sayre syndrome


Type 1 diabetes mellitus

The picture shows an albino fundus.


Albinism represents a group of inherited abnormalities of
melanin synthesis characterised by a congenital
reduction or
absence of melanin pigment, in association with specific
developmental changes in the optic
system resulting from the
hypopigmentation.

Oculocutaneous albinism (OCA) involves two regions of the


body:
1. The skin and hair
2. The optic system including the eye and the optic nerves.

Ocular albinism (OA) has the same changes in the optic system by
reducing mainly the pigment in the retinal
pigment epithelium of
the eye, usually with no clinical difference in the colour of the
skin and hair.

The albino macula is always hypoplastic, and the patient has


reduced acuity and pendular nystagmus.
Strabismus is also
common.

Oculocutaneous albinism

Tyrosinase-related oculocutaneous albinism (OCA1)

This is an autosomal recessive disorder characterised by absence


of pigment in hair, skin, and eyes, and does
not vary with race or
age. Severe nystagmus, photophobia, and reduced visual acuity are
common features.

OCA1 is divided into two types:


1. Type IA, characterised by complete lack of tyrosinase activity
due to production of an inactive enzyme.
2. Type IB (also called yellow OCA), characterised by reduced
activity of tyrosinase.

P-gene related oculocutaneous albinism (OCA2)

OCA2 is the most common type of albinism, and is especially


frequent among African-Americans and Africans.
The estimated
frequency of OCA2 in the African-American population is 1 in
10,000, which contrasts with a
frequency of 1 in 36,000 in
Caucasians.

Chediak-Higashi syndrome

This is autosomal recessive. Patients may have a silvery sheen to


their skin, and blue to brown irises. Patients
have an increased
susceptibility to infection, hepatosplenomegaly, lymphadenopathy
and a predisposition to
development of a lymphoma-like
condition.

Hermansky-Pudlak syndrome

This is autosomal recessive. It is associated with the absence of


platelet-dense bodies, resulting in a loss of
secondary aggregation
of platelets after stimulation, and a predisposition to bruising
and bleeding which can be
severe. There is a higher frequency in
Puerto Rico.

Ocular albinism

X-linked ocular albinism type 1 (OA1)

Although this type of albinism is categorised as a type of ocular


albinism, the melanocytes in the skin and hair
follicles are also
involved. Individuals with OA1 have normal-looking cutaneous
pigment, variable iris pigment,
and reduced or absent retinal
pigment associated with foveal hypoplasia and optic tract
misrouting. The OA1
gene maps to the X chromosome at Xp22.3.

Other options listed in the question.


Abetalipoproteinaemia is associated with retinitis
pigmentosa.

Chronic granulomatous disease presents with recurrent infections


due to an inability to generate the oxidative
burst necessary for
phagocyte killing; it is not associated with any fundal
abnormality.

Cystic fibrosis is associated with recurrent lower respiratory


tract infections but not with fundal abnormality.

Type 1 DM is associated with infections and eye disease - though


diabetic retinopathy does not have this
appearance.

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A 21-year-old woman presented to hospital with vaginal
bleeding.
Test Analysis
She had started her menstrual period the previous day and had
used two boxes of tampons. There was no past
history of
menorrhagia. She was passing bright red fresh blood with no
clots.

On examination she was pale and breathless at rest. Temperature


37.2°C. Her pulse was 110 beats per minute
with blood pressure of
70/40 mmHg. Several large bruises were noted on her arms and
legs.

Investigations showed:

Haemoglobin 53 g/L (115-165)

White cell count 32 ×109/L (4-11)

Platelets 12 ×109/L (150-400)

Fibrin degradation products 1200 mg/L (<100)

The blood film showed a large number of promyelocytes.

What is the most likely diagnosis? Score:

(Please select 1 option) Total Answered:


Acute lymphoblastic leukaemia (ALL)


Acute myeloid leukaemia (AML) type 3
Question Navigator


Idiopathic thrombocytopenic purpura (ITP)
Tags

Meningococcal septicaemia


Toxic shock syndrome

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A 21-year-old woman presented to hospital with vaginal
bleeding.
Answer Statistics
She had started her menstrual period the previous day and had
used two boxes of tampons. There was no past
history of
menorrhagia. She was passing bright red fresh blood with no
clots. Test Analysis
On examination she was pale and breathless at rest. Temperature
37.2°C. Her pulse was 110 beats per minute
with blood pressure of
70/40 mmHg. Several large bruises were noted on her arms and
legs.

Investigations showed:

Haemoglobin 53 g/L (115-165)

White cell count 32 ×109/L (4-11)

Platelets 12 ×109/L (150-400)

Fibrin degradation products 1200 mg/L (<100)

The blood film showed a large number of promyelocytes.

What is the most likely diagnosis?

(Please select 1 option)


Score:
Acute lymphoblastic leukaemia (ALL)
Incorrect answer selected
Total Answered:
Acute myeloid leukaemia (AML) type 3
This is the correct answer


Idiopathic thrombocytopenic purpura (ITP)
Feedback

Meningococcal septicaemia


Toxic shock syndrome Question Navigator

Revision Notes
The patient is bleeding heavily due to a combination of
thrombocytopenia and disseminated intravascular
coagulation
(DIC).
Tags
ALL is not typically associated with DIC and is primarily a
disease of childhood.

The remaining options may all cause DIC, but the presence of
promyelocytes on the blood film is characteristic
of AML type 3 (M3
leukaemia; promyelocytic leukaemia). AML M3 constitutes 10% of all
leukaemias. There is a
predominance of promyelocytes whose
cytoplasm is rich in granules with pro-coagulant activity.

ITP is not associated with DIC.

Meningococcal septicaemia is typically associated with a


purpuric rash and fever (with or without signs of
meningeal
irritation).

Toxic shock syndrome is associated with fever and a widespread


macular 'sunburn' rash that may be associated
with a
retained tampon.

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A 34-year-old man was admitted to hospital with a painful
swollen left leg.
Test Analysis
While in the admissions unit he also developed abdominal pain -
which he said had been recurring over several
months. He had a past
history of a right calf deep vein thrombosis (DVT) six months
previously that had been
treated by anticoagulation with warfarin
for three months. He had noticed that over the past two months his
urine
had been darker than usual in the morning.

Investigations showed:

Haemoglobin 85 g/L (130-180)

White cell count 2.5 ×109/L (4-11)

Platelets 75 ×109/L (150-400)

PT 12 seconds (11.5-15.5)

APTT 35 seconds (30-40)

Serum total bilirubin 29 µmol/L (1-22)


Score:
Serum AST 20 U/L (1-31)

Serum alkaline phosphatase 80 U/L (45-105) Total Answered:

What is the most likely diagnosis?


Question Navigator
(Please select 1 option)

Acute intermittent porphyria Tags

Autoimmune haemolytic anaemia


Factor V Leiden mutation


Paroxysmal nocturnal haemoglobinuria


Protein C deficiency

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Work Smart
A 34-year-old man was admitted to hospital with a painful
swollen left leg.
Answer Statistics
While in the admissions unit he also developed abdominal pain -
which he said had been recurring over several
months. He had a past
history of a right calf deep vein thrombosis (DVT) six months
previously that had been Test Analysis
treated by anticoagulation with warfarin
for three months. He had noticed that over the past two months his
urine
had been darker than usual in the morning.

Investigations showed:

Haemoglobin 85 g/L (130-180)

White cell count 2.5 ×109/L (4-11)

Platelets 75 ×109/L (150-400)

PT 12 seconds (11.5-15.5)

APTT 35 seconds (30-40)

Serum total bilirubin 29 µmol/L (1-22)

Serum AST 20 U/L (1-31)

Serum alkaline phosphatase 80 U/L (45-105)


Score:
What is the most likely diagnosis?
Total Answered:
(Please select 1 option)
Acute intermittent porphyria
Incorrect answer selected Feedback

Autoimmune haemolytic anaemia
Question Navigator

Factor V Leiden mutation

Paroxysmal nocturnal haemoglobinuria


This is the correct answer
Revision Notes

Protein C deficiency

Tags
The diagnosis is paroxysmal nocturnal haemoglobinuria, a
condition in which erythrocytes are abnormally
sensitive to lysis
by complement.

Patients are pancytopenic and have a pro-thrombotic tendency


with DVTs.

Abdominal pain is a common symptom and may be due to small


mesenteric vein thrombi.

Haemolyisis is intermittent - classically nocturnal - giving the


morning urine a typical dark appearance.

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Work Smart MRCP Part 2 Written 2004 December

A 65-year-old man was diagnosed with chronic lymphocytic


leukaemia.
Test Analysis
What antimicrobial prophylaxis should he receive before starting
chemotherapy with fludarabine?

(Please select 1 option)



Aciclovir


Ciprofloxacin


Co-trimoxazole


Intravenous immunoglobulins


Itraconazole

Submit answer
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Score:

Total Answered:

Question Navigator

Tags

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Work Smart MRCP Part 2 Written 2004 December

A 65-year-old man was diagnosed with chronic lymphocytic


leukaemia.
Answer Statistics
What antimicrobial prophylaxis should he receive before starting
chemotherapy with fludarabine?

(Please select 1 option) Test Analysis


Aciclovir
Incorrect answer selected


Ciprofloxacin

Co-trimoxazole
This is the correct answer


Intravenous immunoglobulins


Itraconazole

Fludarabine is a purine analogue that is phosphorylated


intracellularly.

All of the purine analogues cause myelosuppression, but there is


a significantly higher risk of patients developing
Pneumocystis
jirovecii pneumonia while on treatment.

Use of prophylactic co-trimoxazole (Septrin) has dramatically


reduced the frequency of this severe opportunistic
infection in
these patients. Score:

Co-trimoxazole should be continued after chemotherapy until the


CD4 counts exceeds 200 cells/mm3 (0.2 Total Answered:

×109/L).
Feedback

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Work Smart MRCP Part 2 Written 2004 December

A 32-year-old man was admitted to hospital with a fever five


days after receiving a cycle of chemotherapy for
Test Analysis
lymphoma.

On examination, he appeared unwell. He was febrile 39.5°C. Pulse


120 beats per minute, regular with blood
pressure 85/40 mmHg. Heart
sounds were normal with no added sounds or murmurs. His chest was
clear on
auscultation and his abdomen was soft and non-tender.

Investigations revealed:

Haemoglobin 112 g/L (130-180)

White cell count 2.0 ×109/L (4-11)

Neutrophils 0.2 ×109/L (1.5-7)

Lymphocytes 1.6 ×109/L (1.5-4)

Monocytes 0.15 ×109/L (0-0.8)

Eosinophils 0.04 ×109/L (0.04-0.4) Score:


Basophils 0.01 ×109/L (0-0.1) Total Answered:
Platelets 151 ×109/L (150-400)
Question Navigator
What treatment should be started?

(Please select 1 option) Tags



Ceftazidime + ticarcillin/clavulanic acid


Co-trimoxazole


Flucloxacillin + benzylpenicillin


Gentamicin + metronidazole


Piperacillin/tazobactam + gentamicin

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Work Smart MRCP Part 2 Written 2004 December

A 32-year-old man was admitted to hospital with a fever five


days after receiving a cycle of chemotherapy for
Answer Statistics
lymphoma.

On examination, he appeared unwell. He was febrile 39.5°C. Pulse


120 beats per minute, regular with blood Test Analysis
pressure 85/40 mmHg. Heart
sounds were normal with no added sounds or murmurs. His chest was
clear on
auscultation and his abdomen was soft and non-tender.

Investigations revealed:

Haemoglobin 112 g/L (130-180)

White cell count 2.0 ×109/L (4-11)

Neutrophils 0.2 ×109/L (1.5-7)

Lymphocytes 1.6 ×109/L (1.5-4)

Monocytes 0.15 ×109/L (0-0.8)

Eosinophils 0.04 ×109/L (0.04-0.4)

Basophils 0.01 ×109/L (0-0.1)


Score:
Platelets 151 ×109/L (150-400)
Total Answered:
What treatment should be started?

(Please select 1 option) Feedback


Ceftazidime + ticarcillin/clavulanic acid
Incorrect answer selected


Co-trimoxazole Question Navigator

Flucloxacillin + benzylpenicillin
Revision Notes

Gentamicin + metronidazole

Piperacillin/tazobactam + gentamicin
This is the correct answer Tags

Assessment of the febrile patient with neutropenia should


include:
Assess for likely sites of infection from history and clinical
examination and chest x ray.
Comprehensive cultures of blood, urine, sputum, Hickman line
(if present).
If temperature more than 40°C for four to six hours or if the
patient is hypotensive, blind broad-spectrum
antibiotic therapy
should be started (N.B. after taking cultures).
Barrier nursing should be instituted if possible.
Antibiotics should be continued for two to five days after the
fever has settled and the neutrophil count
has recovered.
If fever persists after 48 hours despite antibiotic therapy,
fungal (Aspergillus, Candida,
Pneumocystis) or
viral (Cytomegalovirus or CMV)
infection should be considered.

Although preferred antibiotic regimens for neutropenic fever


vary from centre to centre, all operate on the same
principles. The
aim is to provide broad-spectrum coverage that includes cover for
Pseudomonas aeruginosa.

Dual therapy regimes are usually used. These utilise a


broad-spectrum antibiotic (an antipseudomonal penicillin,

carbapenem or ceftazidime) plus an anti-pseudomonal


aminoglycoside.
Typical regimens consist of anti-pseudomonal aminoglycoside
(Gentamicin, or Amikacin), plus either:
Antipseudomonal penicillin - Piperacillin/tazobactam (Tazocin)
or Ticarcillin/clavulanic acid (Timentin), or
Antipseudomonal carbapenem - Imipenem/cilastatin or
Meropenem.

If infection with Staph. aureus is suspected, the


addition of vancomycin should be considered.

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Work Smart MRCP Part 2 Written 2004 December

A 32-year-old man was referred to clinic with a ten year history


of recurrent chest infections. He was diagnosed
Test Analysis
with common
variable immunodeficiency.

Laboratory investigations showed:


IgG 6.5 g/l (6.0-13.0)

IgA 0.8 g/l (0.8-3.0)

IgM 0.5 g/l (0.4-2.5)

Which of the following options would best prevent recurrent


chest infections?

(Please select 1 option)



Chest physiotherapy and postural drainage


Heart-lung transplantation


Intermittent courses of antibiotics when the patient feels he is developing a chest infection


Intravenous immunoglobulin
Score:

Lifelong prophylactic antibiotics
Total Answered:

Question Navigator
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Work Smart MRCP Part 2 Written 2004 December

A 32-year-old man was referred to clinic with a ten year history


of recurrent chest infections. He was diagnosed
Answer Statistics
with common
variable immunodeficiency.

Laboratory investigations showed: Test Analysis


IgG 6.5 g/l (6.0-13.0)

IgA 0.8 g/l (0.8-3.0)

IgM 0.5 g/l (0.4-2.5)

Which of the following options would best prevent recurrent


chest infections?

(Please select 1 option)


Chest physiotherapy and postural drainage
Incorrect answer selected


Heart-lung transplantation


Intermittent courses of antibiotics when the patient feels he is developing a chest infection

Intravenous immunoglobulin
This is the correct answer


Lifelong prophylactic antibiotics

Score:

Common variable immunodeficiency (CVID) is the most prevalent of


the primary immunodeficiency diseases. Total Answered:
The basic defect in CVID is
a failure of B lymphocyte differentiation into plasma cells that
produce the various
immunoglobulin (Ig) subtypes.
Feedback
Patients with CVID often have marked reduction in serum levels
of both IgG and IgA; approximately 50% of
these patients also have
reduced IgM. However, some patients have normal IgG levels but
functional antibody Question Navigator
deficiency may be present despite normal IgG
subclass levels.

The diagnosis is based on exclusion of known causes of defects


of the humoral immune system. Most cases are Revision Notes
sporadic, although
familial cases exist that have various inheritance modes (including
autosomal dominant with
variable penetrance, autosomal recessive,
and X linked). Tags
Regular intravenous immunoglobulin (IgG) infusions greatly
reduce the frequency of recurrent respiratory tract
infections.

Clinical manifestations of CVID include:

Recurrent infections:

Principally recurrent pyogenic infections of upper and lower


respiratory tract. This is the main clinical
manifestation of CVID.
Symptoms may appear during childhood or, more often, after
adolescence.
Bronchiectasis may develop if optimal therapy is
delayed. Haemophilus influenzae,
Moraxella catarrhalis,
Streptococcus
pneumoniae, and Staphylococcus
aureus are the organisms most commonly involved.
Severe,
recurrent infection with herpes simplex is also common.

Autoimmune diseases:

Patients may develop


Rheumatoid arthritis
Haemolytic anaemia
Thrombocytopenia
Neutropenia
Thyroid abnormalities
Vitiligo
Keratoconjunctivitis sicca.

About 20% of these patients have a severe gastroenteropathy with


severe malabsorption resembling coeliac
sprue, nodular lymphoid
hyperplasia, and chronic inflammatory bowel disease such as
ulcerative colitis and
Crohn's disease. A smaller number of
patients develop
Achlorhydria and pernicious anaemia
Autoimmune hepatitis
Primary biliary cirrhosis
Alopecia totalis
Hyperthyroidism
Vasculitis
Lymphoid interstitial pneumonia.

Lymphoid hyperplasia and granulomatous


diseases:

Atypical lymphoid hyperplasia due to clonal expansion of B or T


lymphocytes has been reported in as many as
one third of patients
with CVID. Granulomas have been reported in approximately 5-10% of
patients with CVID.
Granulomas are indistinguishable from those of
classic sarcoidosis and are found in the
Lung
Liver
Spleen
Conjunctivae.

Predisposition to malignancy:

Patients with CVID have a high risk of developing malignant


neoplasms (most commonly lymphoma, especially
non-Hodgkin's
lymphoma [NHL], and gastrointestinal [GI] carcinoma).

Most lymphomas are of these are of the B cell immunophenotype


and are frequently associated with Epstein-
Barr virus (EBV).
Lymphoma occurs 300 times more frequently in women with CVID than
in affected men.

Other malignancies include


Colon cancer
Breast cancer
Prostate cancer
Ovarian cancer
Oral cancer
Melanoma.

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Work Smart MRCP Part 2 Written 2004 December

A 20-year-old woman presented to hospital with a one week


history of increasing fatigue and exertional
Test Analysis
dyspnoea. She also
complained of the spontaneous appearance of numerous bruises. On
the day of admission
she suddenly developed a high fever and became
drowsy.

On examination she was pale and looked unwell. She was drowsy
but rousable. Temperature 39.5°C, pulse 120
per minute regular,
blood pressure 85/40 mmHg. There was no palpable lymphadenopathy.
Heart sounds were
normal and her chest was clear to auscultation.
Several large bruises were noted on her arms, legs and
abdomen.

Investigations revealed:

Haemoglobin 75 g/L (115-165)

White cell count 40.1 ×109/L (4-11)

Neutrophils 0.1 ×109/L (1.5-7)

Lymphocytes 0.8 ×109/L (1.5-4)

Monocytes 0.1 ×109/L (0-0.8) Score:

Eosinophils 0.098 ×109/L (0.04-0.4) Total Answered:

Basophils 0.002 ×109/L (0-0.1)


Question Navigator
Promyelocytes 39 ×109/L -

Platelets 12 ×109/L (150-400) Tags


PT 25 sec (11.5-15.5)

APTT 50 sec (30-40)

Fibrinogen 0.05 g/L (1.8-5.4)

D-dimer screen 100 mg/L (<0.5)

A high-power picture of her blood film is shown below.


Which of the following chromosomal abnormalities is most likely
to be associated with this patient's illness?

(Please select 1 option)



t(8;14)


t(8;21)


t(9;22)


t(15;17)


t(16;16)

Submit answer
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Work Smart MRCP Part 2 Written 2004 December

A 20-year-old woman presented to hospital with a one week


history of increasing fatigue and exertional
Answer Statistics
dyspnoea. She also
complained of the spontaneous appearance of numerous bruises. On
the day of admission
she suddenly developed a high fever and became
drowsy.
Test Analysis
On examination she was pale and looked unwell. She was drowsy
but rousable. Temperature 39.5°C, pulse 120
per minute regular,
blood pressure 85/40 mmHg. There was no palpable lymphadenopathy.
Heart sounds were
normal and her chest was clear to auscultation.
Several large bruises were noted on her arms, legs and
abdomen.

Investigations revealed:

Haemoglobin 75 g/L (115-165)

White cell count 40.1 ×109/L (4-11)

Neutrophils 0.1 ×109/L (1.5-7)

Lymphocytes 0.8 ×109/L (1.5-4)

Monocytes 0.1 ×109/L (0-0.8)

Eosinophils 0.098 ×109/L (0.04-0.4)


Score:
Basophils 0.002 ×109/L (0-0.1)
Total Answered:
Promyelocytes 39 ×109/L -

Platelets 12 ×109/L (150-400) Feedback


PT 25 sec (11.5-15.5)
Question Navigator
APTT 50 sec (30-40)

Fibrinogen 0.05 g/L (1.8-5.4)


Revision Notes
D-dimer screen 100 mg/L (<0.5)

A high-power picture of her blood film is shown below. Tags


Which of the following chromosomal abnormalities is most likely
to be associated with this patient's illness?

(Please select 1 option)


t(8;14)
Incorrect answer selected


t(8;21)


t(9;22)

t(15;17)
This is the correct answer


t(16;16)

The patient's symptoms and signs are characteristic of acute


promyelocytic leukaemia (AML M3).

Classification and cytogenetics of acute myeloid


leukaemias:

The French-American-British (FAB) classification system of acute


myeloid leukaemias is based on the type of
cell from which the
leukaemia develops and the stage of cellular
differentiation/maturation. Eight categories of
AML are
recognised:
M0 (undifferentiated AML)
M1 (myeloblastic - immature)
M2 (myeloblastic - mature)
M3 (promyelocytic; acute promyelocytic leukaemia; APL)
M4 (myelomonocytic)
M5 (monocytic)
M6 (erythroid)
M7 (megakaryoblastic).

Subtype M2 (acute myeloblastic leukaemia) is the most common


(25% of adult AML) and is associated with a
t(8;21)
translocation.

Subtypes M0, M6, and M7 carry the worst prognosis.

Acute myelomonocytic leukaemia (AML M4) is associated with a


t(16;16) translocation.

Acute promyelocytic leukaemia:

AML M3 (acute promyelocytic leukaemia; APL) is usually


associated with a t(15;17) translocation. Promyelocytes
cytoplasm
is heavily granulated. These granules contain pro-coagulant factors
that render patients with APL
susceptible to the development of
DIC. Despite this, APL has the best prognosis of all the subtypes
of AML.
Unlike the other AML subtypes, APL is treated with
all-trans retinoic acid (ATRA).

The slide in this question shows promyelocytes: the cells have


large, dark-staining nuclei and granular
cytoplasm; the rod-shaped
projections in the cells are Auer rods.

Auer rods are elongated, bluish-red rods composed of fused


lysosomal granules, seen in the cytoplasm of
myeloblasts,
promyelocytes and monoblasts. They may be single or multiple. The
presence of large numbers of
promyelocytes with Auer rods and
cytoplasmic granules in the peripheral circulation is typical of
promyelocytic
leukaemia.

Cytogenetics of other haematological


malignancies:
The acquired chromosomal abnormality 90-95% of patients with
CML is a translocation between
chromosome 9 and 22 (t(9;22)),
resulting in the Philadelphia chromosome.
Ninety percent of cases of Burkitt's lymphoma are due to
the t(8;14) translocation.

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A 65-year-old man was admitted to hospital for a hernia
repair.
Test Analysis
Pre-operative investigations showed:

Haemoglobin 112 g/L (130-180)

MCV 82 fL (80-96)

White cell count 8.1 ×109/L (4-11)

Platelets 345 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags
What is the diagnosis?

(Please select 1 option)



Anaemia of chronic disease


Coeliac disease


Haemoglobin SC disease


Hereditary elliptocytosis


Hyposplenism

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Work Smart
A 65-year-old man was admitted to hospital for a hernia
repair.
Answer Statistics
Pre-operative investigations showed:

Haemoglobin 112 g/L (130-180)


Test Analysis

MCV 82 fL (80-96)

White cell count 8.1 ×109/L (4-11)

Platelets 345 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Feedback
What is the diagnosis?

(Please select 1 option) Question Navigator


Anaemia of chronic disease
Incorrect answer selected


Coeliac disease
Revision Notes


Haemoglobin SC disease
Tags
Hereditary elliptocytosis
This is the correct answer


Hyposplenism

Hereditary elliptocytosis (HE) is an autosomal dominant


condition.

At least four genetic loci have been implicated in the


pathogenesis of the disorder.

Clinical manifestations range from an asymptomatic carriage to


severe haemolytic anaemia. Most patients with
HE or its variants
lead healthy lives.

The hallmark of HE is the presence of cigar-shaped elliptocytes


on the peripheral blood smear. Elliptocytes are
normochromic and
normocytic and range from few to 100% of erythrocytes.

The degree of haemolysis does not correlate with the percentage


of elliptocytes seen in the blood.

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Work Smart
A 62-year-old woman was admitted to hospital for a left knee
replacement.
Test Analysis
Pre-operative investigations showed:

Haemoglobin 141 g/L (115-165)

MCV 92 fL (80-96)

White cell count 6.5 ×109/L (4-11)

Platelets 421 ×109/L (150-400)

Her blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags
Based on these findings, her past medical history is most likely
to include which of the following?

(Please select 1 option)



Alcoholic liver disease


Aortic valve replacement


Bowel resection for Crohn's disease


Febrile illness following travel to Africa


Road traffic accident

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Work Smart
A 62-year-old woman was admitted to hospital for a left knee
replacement.
Answer Statistics
Pre-operative investigations showed:

Haemoglobin 141 g/L (115-165)


Test Analysis

MCV 92 fL (80-96)

White cell count 6.5 ×109/L (4-11)

Platelets 421 ×109/L (150-400)

Her blood film is shown below:

Score:

Total Answered:

Feedback
Based on these findings, her past medical history is most likely
to include which of the following?
Question Navigator
(Please select 1 option)
Alcoholic liver disease
Incorrect answer selected
Revision Notes

Aortic valve replacement


Bowel resection for Crohn's disease Tags

Febrile illness following travel to Africa

Road traffic accident


This is the correct answer

The blood film suggests hyposplenism; of the options given, it


is most likely that the patient's spleen was
removed following
trauma.

The blood film shows variation in erythrocyte size


(anisocytosis) and shape (poikilocytosis).

Howell-Jolly bodies are also seen, which are intracellular


inclusion bodies consisting of remnants of DNA.

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A 74-year-old man was admitted to hospital for a transurethral
resection of the prostate.
Test Analysis
Investigations showed:

Haemoglobin 135 g/L (130-180)

White cell count 32.2 ×109/L (4-11)

Platelets 289 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Question Navigator

Tags

What is the diagnosis?

(Please select 1 option)



Chronic lymphocytic leukaemia


Chronic myeloid leukaemia


Leukoerythroblastic anaemia


Myelodysplastic syndrome


Myelofibrosis

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Work Smart
A 74-year-old man was admitted to hospital for a transurethral
resection of the prostate.
Answer Statistics
Investigations showed:

Haemoglobin 135 g/L (130-180)


Test Analysis

White cell count 32.2 ×109/L (4-11)

Platelets 289 ×109/L (150-400)

His blood film is shown below:

Score:

Total Answered:

Feedback

What is the diagnosis? Question Navigator

(Please select 1 option)


Revision Notes
Chronic lymphocytic leukaemia
Correct


Chronic myeloid leukaemia Tags

Leukoerythroblastic anaemia


Myelodysplastic syndrome


Myelofibrosis

Chronic lymphocytic leukaemia (CLL) is a monoclonal disorder


characterised by a progressive accumulation of
functionally
incompetent lymphocytes.

The cells of origin in the majority of patients with CLL are


clonal B cells arrested in development between pre-B
cells and
mature B cells. Morphologically in the peripheral blood, these
cells resemble mature lymphocytes.

Immunophenotyping typically shows a majority of the population


expressing the following pattern of monoclonal
B cell markers:
Extremely low levels of surface membrane immunoglobulin
(usually IgG or IgM) and either κ or λ (but not
both) light
chains
Expression of one or more B cell associated antigens (CD19,
CD20, CD21, CD23, and CD24) and
Expression of the T cell associated antigen CD5.
There is no one chromosomal change that is specific for CLL,
although certain patterns are frequently seen,
including: trisomy
of chromosomes 12, 3, or 16; del(13q14); del(11q); del(17p);
t(11;14)(q13;q32).

About 25% of patients are asymptomatic, and it is common for


patients to be diagnosed following routine blood
tests. Most
patients with CLL present because they have found painless swollen
lymph nodes, usually in the
cervical chain. However, 5-10% of
patients present with typical B symptoms of lymphoma,
including:
Weight loss more than 10% of body weight within the previous
six months
Fevers more than 38°C for more than two weeks without evidence
of infection
Night sweats without evidence of infection
Profound fatigue.

Less commonly, the disease may manifest as an acquired


immunodeficiency disorder or with autoimmune
phenomena (haemolytic
anaemia, thrombocytopenia).

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A 33-year-old male develops a rash and low grade fever (37.6°C)
21 days post allogeneic bone marrow
Test Analysis
transplant for high risk acute
myeloid leukaemia in first complete remission.

The rash is initially maculopapular affecting palms and soles


but 24 hours later general erythroderma is noted
affecting the
trunk and limbs. His total bilirubin was previously normal but is
now noted to be 40 µmol/L (1-22).
He remains very well in
himself.

What would be your management of this patient at this stage?

(Please select 1 option)



Antibiotics after blood cultures


Antilymphocyte globulin


High dose methylprednisolone


Observation


Prednisolone
Score:

Total Answered:

Submit answer
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Tags

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Work Smart
A 33-year-old male develops a rash and low grade fever (37.6°C)
21 days post allogeneic bone marrow
Answer Statistics
transplant for high risk acute
myeloid leukaemia in first complete remission.

The rash is initially maculopapular affecting palms and soles


but 24 hours later general erythroderma is noted Test Analysis
affecting the
trunk and limbs. His total bilirubin was previously normal but is
now noted to be 40 µmol/L (1-22).
He remains very well in
himself.

What would be your management of this patient at this stage?

(Please select 1 option)


Antibiotics after blood cultures
Incorrect answer selected


Antilymphocyte globulin

High dose methylprednisolone


This is the correct answer


Observation


Prednisolone

This is classical graft versus host disease post bone marrow


transplant.
Score:
Acute graft versus host disease (GVHD) occurs in the first 100
days post transplant with chronic GVHD occurring
Total Answered:
100-300 days post
transplant.

GVHD is graded according to the Seattle system, and each organ


involved is scored (skin, liver and gut). So this
Feedback
patient has two
systems involved.

The candidate should first recognise that this is the diagnosis


and should also know that observation is not an Question Navigator
option for
GVHD.

The standard initial treatment in the acute setting is high dose


methylprednisolone. Action is needed quickly. If Revision Notes
there is no
response then more intensive immunosuppression is needed such as
Campath or antilymphocyte
globulin. Tags
Oral prednisolone is the choice for chronic GVHD.

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A 69-year-old lady is seen in the haematology clinic for the
second time. Her first review was twelve months
Test Analysis
previously.

Her full blood count shows:

Haemoglobin 118 g/L (115 - 165)

White cell count 79 ×109/L (4 - 11)

Neutrophils 4 ×109/L (1.5 - 7)

Lymphocytes 74.5 ×109/L (1.5 - 4)

Monocytes 0.4 ×109/L (0 - 0.8)

Eosinophils 0.05 ×109/L (0.04 - 0.4)

Basophils 0.05 ×109/L (0 - 0.1)

Platelet count 385 ×109/L (150 - 400)


Score:
Her white cell count twelve months previously had been 50
×109/L. She is very well.
Total Answered:
How do you manage her at this stage?

(Please select 1 option) Question Navigator



Chlorambucil
Tags

Fludarabine


Leucapharesis


Observation


Prednisolone

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Work Smart
A 69-year-old lady is seen in the haematology clinic for the
second time. Her first review was twelve months
Answer Statistics
previously.

Her full blood count shows: Test Analysis


Haemoglobin 118 g/L (115 - 165)

White cell count 79 ×109/L (4 - 11)

Neutrophils 4 ×109/L (1.5 - 7)

Lymphocytes 74.5 ×109/L (1.5 - 4)

Monocytes 0.4 ×109/L (0 - 0.8)

Eosinophils 0.05 ×109/L (0.04 - 0.4)

Basophils 0.05 ×109/L (0 - 0.1)

Platelet count 385 ×109/L (150 - 400)

Her white cell count twelve months previously had been 50


×109/L. She is very well.
Score:
How do you manage her at this stage?
Total Answered:
(Please select 1 option)
Chlorambucil
Incorrect answer selected
Feedback

Fludarabine


Leucapharesis
Question Navigator
Observation
This is the correct answer


Prednisolone Revision Notes

Tags
The diagnosis is chronic lymphocytic leukaemia (CLL). This is
easy to make, with an older person with a high
lymphocyte count,
who also had a high lymphocyte count twelve months ago.

This cannot be acute lymphoblastic leukaemia as the patient


would have become seriously unwell in this time
frame and the white
cell differential would not count as lymphocytes but large
undifferentiated cells.

No treatment is necessary and observation only despite the


rising white cell count. There is no cure in this age
group for
CLL, therefore no benefit to early treatment.

The indications for treatment in CLL are:


Lymphocyte doubling time of <6 months
Bone marrow compromise, that is, anaemia, thrombocytopenia,
neutropenia
Autoimmune complications, that is, immune thrombocytopenia or
autoimmune haemolysis
Disabling B symptoms (weight loss or night sweats).

Should any of the above criteria be met at any stage then first
line treatment is with chlorambucil. Prednisolone
can be added to
this but is of no proven benefit, although it sometimes makes the
patients feel better.

Fludarabine is second line treatment.

Leucapheresis, the removal of white cells from the peripheral


blood via a cell separation machine, is not used in
CLL. It is
sometimes used in chronic myeloid leukaemia and acute myeloid
leukaemia with high counts but only
if the patient develops
leucostatic signs and symptoms.

Reference:

British Committee for Standards in Haematology (BCSH). Guidelines on the diagnosis, investigation and

management of Chronic Lymphocytic Leukaemia.

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A 33-year-old lady is admitted to the gynaecology ward at 24
weeks gestation in her first pregnancy.
Test Analysis
She had seen her midwife and complained of feeling generally not
well. Her husband reported that she has
become very vague and
disorientated. The midwife found her blood pressure to be 140/89
mmHg; her booking
blood pressure was 120/80 mmHg.

On admission her blood tests are as follows:

Haemoglobin 79 g/L (115-165)

White cell count 11 ×109/L (4-11) Normal differential

Platelets 30 ×109/L (150-400)

MCV 103 fL (80-96)

Reticulocyte count 12.5% -

Blood film shows red cell fragmentation with thrombocytopenia


and platelet anisocytosis. There is
polychromasia.
Score:
Serum lactate dehyrogenase 1112 U/L (10-250)
Total Answered:
Serum sodium 140 mmol/L (137-144)

Serum potassium 3.7 mmol/L (3.5-4.9) Question Navigator


Serum urea 15.2 mmol/L (2.5-7.5)

Serum creatinine 200 µmol/L (60-110) Tags


 

Serum total bilirubin 73 µmol/L (1-22)

Serum alanine aminotranseferase 40 U/L (5-35)

Serum alkaline phosphatase 160 U/L (45-105)

Coagulation screen Normal -

Fibrinogen 5.3 g/L (1.8-5.4)

What treatment would you advise the obstetric team to give to


this lady?

(Please select 1 option)



Fresh frozen plasma transfusion


Haemodialysis


None


Plasma exchange


Platelet transfusion

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Work Smart
A 33-year-old lady is admitted to the gynaecology ward at 24
weeks gestation in her first pregnancy.
Answer Statistics
She had seen her midwife and complained of feeling generally not
well. Her husband reported that she has
become very vague and
disorientated. The midwife found her blood pressure to be 140/89
mmHg; her booking Test Analysis
blood pressure was 120/80 mmHg.

On admission her blood tests are as follows:

Haemoglobin 79 g/L (115-165)

White cell count 11 ×109/L (4-11) Normal differential

Platelets 30 ×109/L (150-400)

MCV 103 fL (80-96)

Reticulocyte count 12.5% -

Blood film shows red cell fragmentation with thrombocytopenia


and platelet anisocytosis. There is
polychromasia.

Serum lactate dehyrogenase 1112 U/L (10-250)

Serum sodium 140 mmol/L (137-144) Score:

Serum potassium 3.7 mmol/L (3.5-4.9) Total Answered:


Serum urea 15.2 mmol/L (2.5-7.5)

Serum creatinine 200 µmol/L (60-110) Feedback


 

Serum total bilirubin 73 µmol/L (1-22) Question Navigator


Serum alanine aminotranseferase 40 U/L (5-35)
Revision Notes
Serum alkaline phosphatase 160 U/L (45-105)

 
Tags
Coagulation screen Normal -

Fibrinogen 5.3 g/L (1.8-5.4)

What treatment would you advise the obstetric team to give to


this lady?

(Please select 1 option)


Fresh frozen plasma transfusion
Incorrect answer selected


Haemodialysis


None

Plasma exchange
This is the correct answer


Platelet transfusion

This lady has features of thrombotic thrombocytopenia purpura


(TTP).

First this requires observation that she has a microangiopathic


haemolytic anaemia (MAHA) with
thrombocytopenia, low haemoglobin
with a raised reticulocyte count, raised lactate dehydrogenase
(LDH) and
bilirubin.

The film shows fragmentation.

In TTP there is typically a classical pentad of:


1. MAHA
2. Severe thrombocytopenia
3. Neurological impairment
4. Renal impairment
5. Fever.

We know she has at least four of these.

The differential diagnoses of a MAHA with thrombocytopenia in


pregnancy are as follows:
TTP
Disseminated intravascular coagulation (DIC)
Pre-eclampsia
Haemolytic uraemic syndrome (HUS)
HELLP - haemolysis elevated liver enzymes and low
platelets.

Against DIC, the fibrinogen is entirely normal!

In pre-eclampsia the platelet count does not tend to be as low,


nor such a degree as haemolysis. It tends to
occur later in
pregnancy, although can occur early. TTP classically occurs
earlier.

HUS is very similar to TTP, but classically occurs post partum -


after an otherwise uneventful pregnancy, a post-
delivery woman
suddenly becomes oliguric. You would also expect more severe renal
function derangement.

HELLP - you would expect more significant derrangemnt of liver


functiion tests (LFTs) and a later pregnancy
complication.

The only beneficial treatment for acquired TTP is plasma


exchange, daily with fresh frozen plasma (FFP). In
addition, three
days of intravenous steroids are also recommended in the current
guidelines.

FFP can be used to stabilise the patient whilst the definitive


treatment can be arranged.

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Work Smart MRCP Part 2 Written 2005 July

An 18-year-old man was admitted to casualty at 3 am having


collapsed at a rave party.
Test Analysis
A friend, who accompanied him to hospital, said that the patient
had been entirely well earlier in the evening and
had been dancing
most of the night. He reported that his friend seldom drank
alcohol, although there was
alcohol available at the party venue.
He did not know whether his friend had taken any drugs.

Investigations revealed:

Haemoglobin 89 g/L (130 - 180)

White cell count 25.0 ×109/L (4 - 11)

Neutrophils 21.7 ×109/L (1.5 - 7.0)

Lymphocytes 2.0 ×109/L (1.5 - 4.0)

Monocytes 0.8 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1) Score:

Platelets 27 ×109/L (150 - 400)


Total Answered:

Reticulocyte count 10%


Question Navigator
Prothrombin time 32 secs (11.5 - 15.5)

Activated partial thromboplastin time 82 secs (30 - 40) Tags


Fibrinogen 0.3 g/L (1.8 - 5.4)

D-Dimer screen 2.6 mg/L (<0.5)

Serum sodium 137 mmol/L (137 - 144)

Serum potassium 6.3 mmol/L (3.5 - 4.9)

Serum urea 17.5 mmol/L (2.5 - 7.5)

Serum creatinine 200 µmol/L (60 - 110)

Lactate dehydrogenase 525 U/L (10 - 250)

A blood film showed red cell fragmentation with polychromasia,


toxic granulation of neutrophils and platelet
anisocytosis.

What is the diagnosis?

(Please select 1 option)



Autoimmune haemolysis


Autoimmune thrombocytopenia


Disseminated intravascular coagulation (DIC)


Drug-induced haemolysis


Thrombotic thrombocytopenic purpura (TTP)

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Work Smart MRCP Part 2 Written 2005 July

An 18-year-old man was admitted to casualty at 3 am having


collapsed at a rave party.
Answer Statistics
A friend, who accompanied him to hospital, said that the patient
had been entirely well earlier in the evening and
had been dancing
most of the night. He reported that his friend seldom drank
alcohol, although there was Test Analysis
alcohol available at the party venue.
He did not know whether his friend had taken any drugs.

Investigations revealed:

Haemoglobin 89 g/L (130 - 180)

White cell count 25.0 ×109/L (4 - 11)

Neutrophils 21.7 ×109/L (1.5 - 7.0)

Lymphocytes 2.0 ×109/L (1.5 - 4.0)

Monocytes 0.8 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)

Platelets 27 ×109/L (150 - 400)


Score:
Reticulocyte count 10%
Total Answered:
Prothrombin time 32 secs (11.5 - 15.5)

Activated partial thromboplastin time 82 secs (30 - 40) Feedback


Fibrinogen 0.3 g/L (1.8 - 5.4)

D-Dimer screen 2.6 mg/L (<0.5)


Question Navigator

Serum sodium 137 mmol/L (137 - 144) Revision Notes


Serum potassium 6.3 mmol/L (3.5 - 4.9)

Serum urea 17.5 mmol/L (2.5 - 7.5) Tags


Serum creatinine 200 µmol/L (60 - 110)

Lactate dehydrogenase 525 U/L (10 - 250)

A blood film showed red cell fragmentation with polychromasia,


toxic granulation of neutrophils and platelet
anisocytosis.

What is the diagnosis?

(Please select 1 option)


Autoimmune haemolysis
Incorrect answer selected


Autoimmune thrombocytopenia

Disseminated intravascular coagulation (DIC)


This is the correct answer


Drug-induced haemolysis


Thrombotic thrombocytopenic purpura (TTP)
There is evidence of a microangiopathic haemolytic anaemia with
thrombocytopenia (low haemoglobin, high
reticulocyte count with
polychromasia, elevated serum lactate dehydrogenase [LDH], and low
platelets). Given
this picture, the only reasonable options are DIC
or TTP.

In TTP, the coagulation and D-dimers are usually normal: this


can be used to differentiate TTP from DIC.

In DIC there is the microangiopathic haemolytic anaemia with


thrombocytopenia, but the coagulation is abnormal
due to the
consumptive process - classically hypofibrinogenaemia develops.

There are a large number of causes of DIC. One such cause is


tissue damage, causing release of tissue factor,
such as might
occur in trauma, thermal injury - hypo/hyperthermia or
rhabdomyolysis.

The classical pentad of TTP comprises:


1. Microangiopathic haemolytic anaemia
2. Severe thrombocytopenia
3. Neurological involvement
4. Renal impairment
5. Fever

As an aide-memoire, this can be remembered as:


P - Pyrexia
E - Endothelial damage
N - Neurological abnormality
T - Thrombocytopenia
A - Anaemia (MAHA)
D - Damage to kidney.

Not all of these features need to be present in TTP.

The likelihood is this young man has rhabdomyolysis triggered by


recent ecstasy intake. Ecstasy is well-known to
be used at raves
and is more likely to have a toxic effect when individuals are
dehydrated.

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Work Smart MRCP Part 2 Written 2005 July

A 23-year-old male had a bone marrow transplant from his sister


for acute myeloid leukaemia.
Test Analysis
Two months later he presented with a rash and general malaise
with lethargy and poor appetite. On further
questioning the patient
revealed that he had had episodes of green-coloured diarrhoea for
several weeks. His
medications included cyclosporin, penicillin V,
co-trimoxazole and aciclovir. He was last given a blood
component
transfusion four weeks previously.

On examination, a diffuse macular rash was visible, mainly


affecting the palms of the hands and soles of the feet.
His pulse
was 110 beats per minute and regular. Blood pressure 105/60 mmHg.
Heart sounds were normal and
the chest was clear. His abdomen was
soft, with tenderness in the right upper quadrant. No organs were

palpable. Bowel sounds were active. Neurological examination was


normal.

Investigations revealed:

Haemoglobin 129 g/L (130-180)

MCV 94 fL (80-96)

White cell count 3.65 ×109/L (4-11)


Score:
Neutrophils 1.7 ×109/L (1.5-7)
Total Answered:
Lymphocytes 1.0 ×109/L (1.5-4)

Monocytes 0.8 ×109/L (0-0.8) Question Navigator


Eosinophils 0.05 ×109/L (0.04-0.4)
Tags
Basophils 0.1 ×109/L (0-0.1)

Platelets 147 ×109/L (150-400)

Serum albumin 35 g/L (37-49)

Serum total bilirubin 50 µmol/L (1-22)

Serum alanine transferase 35 U/L (5-35)

Serum alkaline phosphatase 120 U/L (45-105)

Serum gamma glutamyl transferase 35 U/L (4-35)

What is the diagnosis?

(Please select 1 option)



Acute graft versus host disease


Clostridium difficile infection


Cytomegalovirus (CMV) infection


Side effect of immunosuppression


Transfusion-associated graft versus host disease (GVHD)

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Work Smart MRCP Part 2 Written 2005 July

A 23-year-old male had a bone marrow transplant from his sister


for acute myeloid leukaemia.
Answer Statistics
Two months later he presented with a rash and general malaise
with lethargy and poor appetite. On further
questioning the patient
revealed that he had had episodes of green-coloured diarrhoea for
several weeks. His Test Analysis
medications included cyclosporin, penicillin V,
co-trimoxazole and aciclovir. He was last given a blood
component
transfusion four weeks previously.

On examination, a diffuse macular rash was visible, mainly


affecting the palms of the hands and soles of the feet.
His pulse
was 110 beats per minute and regular. Blood pressure 105/60 mmHg.
Heart sounds were normal and
the chest was clear. His abdomen was
soft, with tenderness in the right upper quadrant. No organs were

palpable. Bowel sounds were active. Neurological examination was


normal.

Investigations revealed:

Haemoglobin 129 g/L (130-180)

MCV 94 fL (80-96)

White cell count 3.65 ×109/L (4-11)

Neutrophils 1.7 ×109/L (1.5-7)

Lymphocytes 1.0 ×109/L (1.5-4) Score:

Monocytes 0.8 ×109/L (0-0.8) Total Answered:

Eosinophils 0.05 ×109/L (0.04-0.4)


Feedback
Basophils 0.1 ×109/L (0-0.1)

Platelets 147 ×109/L (150-400) Question Navigator


 
Revision Notes
Serum albumin 35 g/L (37-49)

Serum total bilirubin 50 µmol/L (1-22)


Tags
Serum alanine transferase 35 U/L (5-35)

Serum alkaline phosphatase 120 U/L (45-105)

Serum gamma glutamyl transferase 35 U/L (4-35)

What is the diagnosis?

(Please select 1 option)


Acute graft versus host disease
Correct


Clostridium difficile infection


Cytomegalovirus (CMV) infection


Side effect of immunosuppression


Transfusion-associated graft versus host disease (GVHD)

This case is describing classic acute graft versus host disease


(GVHD).
The key to this is that the patient has a multi-system disease.
Organs usually involved in GVHD are skin, liver
and gut. The rash
on the palms and soles is the classical textbook description of the
skin involvement in GVHD.

The most marked abnormality on the liver function tests (LFTs)


is the raised bilirubin. There is also abnormal
coloured
diarrhoea.

The candidate should recognise that the full blood count (FBC)
is essentially normal for a patient post recent
bone marrow
transplant (BMT).

CMV infection can cause a hepatitic picture with gut involvement


and a non-specific rash, but this is usually
associated with
myelosuppression. In this case the FBC is very good for someone
post BMT.

Cyclosporin does not usually cause these symptoms, signs and


biochemical abnormalities.

Transfusion associated GVHD is a rare but usually fatal


complication seen post BMT. It is due to donor
lymphocytes in
transfused cellular components, for example, blood, platelets.
These donor lymphocytes
recognise the recipient as foreign, and as
the recipient is immunocompromised due to the recent BMT they

cannot set up a reaction to destroy these incoming lymphocytes.

In this case the recipient is the host and the incoming


lymphocytes are the graft. The lymphocytes cause bone
marrow
failure, liver dysfunction and gastrointestinal symptoms. There is
no cure, only preventative measures,
in that all cellular blood
components given to BMT recipients need to be irradiated, this
limits the functional
activity of the lymphocytes.

It usually occurs about 14 days after the affected transfusion.


In this case the FBC result is not in keeping at all
and the timing
is wrong.

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Work Smart MRCP Part 2 Written 2005 July

An 82-year-old lady presented to her GP with a two-month history


of severe back pain. She had not been feeling
Test Analysis
well for several
weeks and had lost weight, though was unsure how much.

Her past medical history included angina, which was


well-controlled on atenolol 100 mg daily and isosorbide
mononitrate
10 mg twice daily. She was an ex-smoker of 40 cigarettes per day,
having given up smoking eight
years previously.

She lived alone in a warden-monitored flat and usually managed


to cook and clean for herself - but her ability to
perform her
daily chores had become increasingly difficult over the past week
because of the severity of the
pain. For the past two days she had
not managed to get out of bed. Her husband had died three years

previously of lung cancer. She had two children who lived several
hours drive away.

Because of the severity of the pain and her inability to cope at


home, she was admitted to hospital.

Investigations revealed:
Haemoglobin 91 g/L (115-165)

MCV 92 fL (80-96)

4.0 ×109/L Score:


White cell count (4-11)
Total Answered:
Platelets 87 ×109/L (150-400)

Serum sodium 137 mmol/L (137-144)


Question Navigator
Serum potassium 4.9 mmol/L (3.5-4.9)

Serum urea 12.4 mmol/L (2.5-7.5)


Tags
Serum creatinine 160 µmol/L (60-110)

Serum corrected calcium 2.5 mmol/L (2.2-2.6)

Serum albumin 37 g/L (37-49)

Serum phosphate 1.5 mmol/L (0.8-1.4)

Serum total protein 96 g/L (61-76)

Serum IgG 32 g/L (6.0-13.0)

Serum IgA 0.2 g/L (0.8-3.0)

Serum IgM 0.1 g/L (0.4-2.5)

Serum Electrophoresis: Single band in gamma region typed as IgG


Kappa. Plain x ray showed collapse of L1
and L2 vertebrae.

Which of the following would be the most suitable therapy to


control this patient's back pain?

(Please select 1 option)



Bisphosphonate


Dexamethasone


Melphalan


Non steroidal Anti-inflammatory agent


Radiotherapy

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Work Smart MRCP Part 2 Written 2005 July

An 82-year-old lady presented to her GP with a two-month history


of severe back pain. She had not been feeling
Answer Statistics
well for several
weeks and had lost weight, though was unsure how much.

Her past medical history included angina, which was


well-controlled on atenolol 100 mg daily and isosorbide Test Analysis
mononitrate
10 mg twice daily. She was an ex-smoker of 40 cigarettes per day,
having given up smoking eight
years previously.

She lived alone in a warden-monitored flat and usually managed


to cook and clean for herself - but her ability to
perform her
daily chores had become increasingly difficult over the past week
because of the severity of the
pain. For the past two days she had
not managed to get out of bed. Her husband had died three years

previously of lung cancer. She had two children who lived several
hours drive away.

Because of the severity of the pain and her inability to cope at


home, she was admitted to hospital.

Investigations revealed:
Haemoglobin 91 g/L (115-165)

MCV 92 fL (80-96)

White cell count 4.0 ×109/L (4-11)

Platelets 87 ×109/L (150-400)


Score:
Serum sodium 137 mmol/L (137-144)
Total Answered:
Serum potassium 4.9 mmol/L (3.5-4.9)

Serum urea 12.4 mmol/L (2.5-7.5) Feedback


Serum creatinine 160 µmol/L (60-110)

Serum corrected calcium 2.5 mmol/L (2.2-2.6) Question Navigator


Serum albumin 37 g/L (37-49)

Serum phosphate 1.5 mmol/L (0.8-1.4) Revision Notes


Serum total protein 96 g/L (61-76)
Tags
Serum IgG 32 g/L (6.0-13.0)

Serum IgA 0.2 g/L (0.8-3.0)

Serum IgM 0.1 g/L (0.4-2.5)

Serum Electrophoresis: Single band in gamma region typed as IgG


Kappa. Plain x ray showed collapse of L1
and L2 vertebrae.

Which of the following would be the most suitable therapy to


control this patient's back pain?

(Please select 1 option)


Bisphosphonate
Incorrect answer selected


Dexamethasone


Melphalan


Non steroidal Anti-inflammatory agent

Radiotherapy
This is the correct answer
The history and the results are suggestive of multiple myeloma.
She has impaired renal function, compromised
bone marrow function,
raised total protein with an elevated serum IgG with a paraprotein
on electrophoresis. Her
IgA and IgM are low. The x-ray is reported
as collapse which in the context of myeloma is likely to be related
to
a lytic lesion.

Radiotherapy is extremely effective as pain control in this


situation and would be the ideal choice. NSAIDs
should be avoided
in a patient with myeloma and impaired renal function. Melphalan
and dexamethasone both
have a place in the treatment of myeloma but
would not be of use as pain control. A patient with myeloma, with

or without evidence of bony disease should be on a bisphosphonate,


which is good for bony pain symptoms as
well; but not really acute
severe back pain

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Work Smart MRCP Part 2 Written 2005 July

An 18-year-old lady was diagnosed with acute lymphoblastic


leukaemia. As part of the chemotherapy regimen,
Test Analysis
she required weekly
lumbar punctures to administer intrathecal chemotherapy. When she
attended the day unit
for her third course of treatment, her
platelet count was found to be 25 ×109/L (150-400
×109/L).

What is the best course of action?

(Please select 1 option)



Start tranexamic acid and perform the lumbar puncture after six hours.


Use DDAVP before performing the lumbar puncture.


Delay the lumbar puncture until the platelet count improves


Give a platelet transfusion before performing the lumbar puncture


Proceed with the lumbar puncture as benefit of receiving chemotherapy outweighs the risks.

Score:
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Total Answered:

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Work Smart MRCP Part 2 Written 2005 July

An 18-year-old lady was diagnosed with acute lymphoblastic


leukaemia. As part of the chemotherapy regimen,
Answer Statistics
she required weekly
lumbar punctures to administer intrathecal chemotherapy. When she
attended the day unit
for her third course of treatment, her
platelet count was found to be 25 ×109/L (150-400
×109/L).
Test Analysis
What is the best course of action?

(Please select 1 option)


Start tranexamic acid and perform the lumbar puncture after six hours.
Incorrect answer selected


Use DDAVP before performing the lumbar puncture.


Delay the lumbar puncture until the platelet count improves

Give a platelet transfusion before performing the lumbar puncture


This is the correct answer


Proceed with the lumbar puncture as benefit of receiving chemotherapy outweighs the risks.

This question relates to safety for different procedures when


thrombocytopenic. In general a platelet count of 10-
20
×109 /L is safe for most procedures. The exceptions to
this are major surgey and procedures involving the
CNS and eyes. In
the latter cases, the platelet count should be above 50
×109/L.
Score:
Clearly, in this case the platelet count is too low to proceed
safely with the lumbar puncture. Giving one unit of
platelets
pre-procedure should correct this to a safe level, although a
repeat FBC would be recommended. Total Answered:

As she is on a chemotherapy regimen it is likely that it will be


a while before the platelet count will improve
spontaneously. It is
important not to delay any chemotherapy as this could adversely
affect the outcome. Feedback

Tranexamic acid is an antifibrinolytic agent. As such, its


effectiveness relies on the presence of adequate
Question Navigator
numbers of
platelets in order to form a clot. DDAVP is of use in some patients
with platelet function disorders but
not for a reduced platelet
count.
Revision Notes

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Work Smart MRCP Part 2 Written 2005 July

A 53-year-old gentleman was admitted to hospital after


presenting to his GP complaining of feeling lethargic and
Test Analysis
generally
unwell.

Despite having a normal appetite, he had lost two stones in


weight in six weeks. He also reported sweating
profusely at night
and his wife said that she often had to change the sheets because
they were drenched. He
also has some upper abdominal
discomfort.

On examination he looked thin and unwell. His pulse was 90 beats


per minute in sinus rhythm with blood
pressure 145/80 mmHg. A short
systolic murmur was audible at the lower left sternal edge. His
chest was clear.
His abdomen was soft, with slight tenderness in
the epigastrium and central abdomen. The spleen was palpable
7 cm
below the left costal margin.

Investigations revealed:

Haemoglobin 97 g/L (130-180)

White cell count 17.4 ×109/L (4-11)

Neutrophils 14.0 ×109/L (1.5-7)


Score:
Lymphocytes 1.5 ×109/L (1.5-4)
Total Answered:
Monocytes 0.8 ×109/L (0-0.8)

Basophils 0.7 ×109/L (0-0.1)


Question Navigator

Eosinohils 0.4 ×109/L (0.04-0.4)


Tags
Platelets 550 ×109/L (150-400)

The blood film showed left-shifted neutrophils with numerous


myelocytes present. Occasional promyelocytes
were seen, but no
blasts. There were also a number of nucleated red blood cells as
well as a large number of
tear drop red cells. There was a
thrombocytosis with platelet anisocytosis.

What investigation should be performed next?

(Please select 1 option)



Cytogenetic analysis of the patient’s bone marrow


Immunophenotyping of peripheral blood


Lactate dehydrogenase


Neutrophil Alkaline Phosphatase score


Ultrasound scan of abdomen

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Work Smart MRCP Part 2 Written 2005 July

A 53-year-old gentleman was admitted to hospital after


presenting to his GP complaining of feeling lethargic and
Answer Statistics
generally
unwell.

Despite having a normal appetite, he had lost two stones in


weight in six weeks. He also reported sweating Test Analysis
profusely at night
and his wife said that she often had to change the sheets because
they were drenched. He
also has some upper abdominal
discomfort.

On examination he looked thin and unwell. His pulse was 90 beats


per minute in sinus rhythm with blood
pressure 145/80 mmHg. A short
systolic murmur was audible at the lower left sternal edge. His
chest was clear.
His abdomen was soft, with slight tenderness in
the epigastrium and central abdomen. The spleen was palpable
7 cm
below the left costal margin.

Investigations revealed:

Haemoglobin 97 g/L (130-180)

White cell count 17.4 ×109/L (4-11)

Neutrophils 14.0 ×109/L (1.5-7)

Lymphocytes 1.5 ×109/L (1.5-4)

0.8 ×109/L
Score:
Monocytes (0-0.8)
Total Answered:
Basophils 0.7 ×109/L (0-0.1)

Eosinohils 0.4 ×109/L (0.04-0.4)


Feedback
Platelets 550 ×109/L (150-400)

Question Navigator
The blood film showed left-shifted neutrophils with numerous
myelocytes present. Occasional promyelocytes
were seen, but no
blasts. There were also a number of nucleated red blood cells as
well as a large number of
tear drop red cells. There was a
thrombocytosis with platelet anisocytosis. Revision Notes

What investigation should be performed next?


Tags
(Please select 1 option)
Cytogenetic analysis of the patient’s bone marrow
Correct


Immunophenotyping of peripheral blood


Lactate dehydrogenase


Neutrophil Alkaline Phosphatase score


Ultrasound scan of abdomen

The candidate should recognise the abnormalities given in the


question:
anaemia
high WBC
thrombocytosis
a leucoerythroblastic blood film with characteristic tear drop
cells, and
the presence of 'B'symptoms (weight loss and night
sweats with splenomegaly).

The differential diagnosis in this case is between the


myeloproliferative disorders Chronic Myeloid Leukaemia
(CML),
Myelofibrosis and Essential Thrombocythaemia (ET).

CML and myelofibrosis can be very difficult to distinguish in


the early stages on morphology alone or on clinical
findings and
the most useful test is cytogenetic analysis - most cases of CML
are usually associted with BCR-
ABL translocation, whereas this is
not seen in myelofibrosis or ET (although other cytogenetic
abnormalities are
seen). It is most useful to do cytogenetic
analysis on a bone marrow sample rather than peripheral blood

because the cellularity tends to be greater in the BM, giving lower


failure rates of the test. A bone marrow is
indicated anyway
because if this patient was BCR-ABL negative on the peripheral
blood a bone marrow would
be necessary to further facilitate
diagnosis.

LDH, NAP and USS are not going to help with making the
diagnosis, neither is immunophenotyping, as there are
no blasts in
the blood and this is not an acute leukaemia.

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Work Smart MRCP Part 2 Written 2005 July

A 62-year-old man attended the outpatient clinic for a follow up


appointment. He had a history of two venous
Test Analysis
thromboembolic events
and wanted advice regarding his treatment with warfarin.

He had been diagnosed with an ilio-femoral deep vein thrombosis


two years previously and had been treated
with a six month course
of warfarin. A thrombophilia screen, performed two months after
stopping the warfarin,
was negative.

Six months after stopping warfarin he presented to hospital with


left-sided pleuritic chest pain. His ECG was
normal but a CT
pulmonary angiogram showed a pulmonary embolus. On this occasion
there were no obvious
risk factors, other than his previous event.
He was recommenced on warfarin.

At the time of his follow up outpatient appointment he was


approaching completion of six months of treatment.
During his
hospital admission, he had been advised that he should receive
lifelong treatment with warfarin.
However, he expressed concern
about the risk of bleeding while on warfarin.

On further questioning, he reveals that he has had two


admissions to hospital with episodes of bleeding in the
past three
months. On the first occasion he had a spontaneous epistaxis. The
second admission was for
bleeding from a scalp wound after he hit
his head accidentally on the bathroom cupboard. On both occasions
his
Score:
INR was over 8 and he had been admitted until the INR returned
to normal.
Total Answered:
What is the best course of action?

(Please select 1 option)


Question Navigator

Continue warfarin and check compliance and monitoring of INR


Stop warfain Tags

Stop warfarin and give long term low molecular weight heparin


Stop warfarin and implant an inferior vena caval filter


Stop warfarin and receive low molecular weight heparin for long journeys

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Work Smart MRCP Part 2 Written 2005 July

A 62-year-old man attended the outpatient clinic for a follow up


appointment. He had a history of two venous
Answer Statistics
thromboembolic events
and wanted advice regarding his treatment with warfarin.

He had been diagnosed with an ilio-femoral deep vein thrombosis


two years previously and had been treated Test Analysis
with a six month course
of warfarin. A thrombophilia screen, performed two months after
stopping the warfarin,
was negative.

Six months after stopping warfarin he presented to hospital with


left-sided pleuritic chest pain. His ECG was
normal but a CT
pulmonary angiogram showed a pulmonary embolus. On this occasion
there were no obvious
risk factors, other than his previous event.
He was recommenced on warfarin.

At the time of his follow up outpatient appointment he was


approaching completion of six months of treatment.
During his
hospital admission, he had been advised that he should receive
lifelong treatment with warfarin.
However, he expressed concern
about the risk of bleeding while on warfarin.

On further questioning, he reveals that he has had two


admissions to hospital with episodes of bleeding in the
past three
months. On the first occasion he had a spontaneous epistaxis. The
second admission was for
bleeding from a scalp wound after he hit
his head accidentally on the bathroom cupboard. On both occasions
his
INR was over 8 and he had been admitted until the INR returned
to normal.

What is the best course of action? Score:


(Please select 1 option) Total Answered:
Continue warfarin and check compliance and monitoring of INR
Correct


Stop warfain Feedback

Stop warfarin and give long term low molecular weight heparin
Question Navigator

Stop warfarin and implant an inferior vena caval filter


Stop warfarin and receive low molecular weight heparin for long journeys
Revision Notes

This is a difficult question. The greatest risk factor for


recurrence of a venous thromboembolism (VTE) is a Tags
previous episode.
This gentleman is at high risk of having a recurrence if he stops
warfarin - he has had two
separate thromboembolic episodes.

Although the first episode had an attributable transient risk


factor, the second event did not. Both episodes of
bleeding were
associated with over-anticoagulation, suggesting either infrequent
attendance, or a need for more
regular monitoring. This should be
discussed in detail with the patient to ensure that he understands
the need to
maintain a stable INR and to make sure that there are
no extraneous factors (for example, taking herbal
medicines or
antibiotics) affecting his anticoagulation. He should continue
lifelong warfarin with a target INR of
2.5.

Two recent trials (PREVENT and ELATE) both looked at continuing


warfarin to prevent recurrence of VTE. The
ELATE trial also looked
at two target ranges of INR. The British Committee for Standards in
Haematology have
since recommended a target INR of 2.5 for long
term oral anticoagulant therapy for secondary prevention of

VTE.

For those patients who are at high risk of bleeding which might
ordinarily result in treatment being stopped may
be treated to a
lower target INR (1.75). Such patients might be those who have
already suffered a major bleed
and in whom the risk of bleeding has
not altered, or those patients with repeated episodes of
over-
anticoagulation requiring reversal with vitamin K or
coagulation factor replacement on more than one occasion.
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An 82-year-old lady was admitted to hospital with fever and
confusion. She had been found collapsed at home
Test Analysis
by her son, who
reported that she was usually fit and well and coped well on her
own at home.

She had a past history of angina, which was well-controlled on a


beta-blocker and oral nitrate and she also
suffered from
osteoarthritis.

On examination she looked pale and unwell and smelled strongly


of stale urine. Her temperature was 38.1°C.
She was disoriented in
time, place and person. Her blood pressure was 80/45 mmHg with
pulse 110 beats per
minute and regular.

There were widespread petechiae on the limbs. Her respiratory


rate was measured at 28 breaths per minute. Her
heart sounds were
normal and the chest clear. Her abdomen was soft, but she was very
tender in the
suprapubic region and a mass was felt rising from the
pelvic brim.

After taking blood, it was noted that there was continued


bleeding from the venous puncture site. A urinary
catheter was
inserted and yielded 1500 ml of cloudy yellow offensive-smelling
urine.

Which of the following statements is true?


Score:
(Please select 1 option)

Circulating levels of activated protein C (aPC) will be reduced Total Answered:


Levels of D-dimer will be reduced
Question Navigator

Levels of fibrin degradation products (FDPs) will be reduced


The activated partial thromboplastin time (APTT) will be below the normal range Tags

The platelet count is likely to be elevated

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An 82-year-old lady was admitted to hospital with fever and
confusion. She had been found collapsed at home
Answer Statistics
by her son, who
reported that she was usually fit and well and coped well on her
own at home.

She had a past history of angina, which was well-controlled on a


beta-blocker and oral nitrate and she also Test Analysis
suffered from
osteoarthritis.

On examination she looked pale and unwell and smelled strongly


of stale urine. Her temperature was 38.1°C.
She was disoriented in
time, place and person. Her blood pressure was 80/45 mmHg with
pulse 110 beats per
minute and regular.

There were widespread petechiae on the limbs. Her respiratory


rate was measured at 28 breaths per minute. Her
heart sounds were
normal and the chest clear. Her abdomen was soft, but she was very
tender in the
suprapubic region and a mass was felt rising from the
pelvic brim.

After taking blood, it was noted that there was continued


bleeding from the venous puncture site. A urinary
catheter was
inserted and yielded 1500 ml of cloudy yellow offensive-smelling
urine.

Which of the following statements is true?

(Please select 1 option)


Circulating levels of activated protein C (aPC) will be reduced
Correct
Score:

Levels of D-dimer will be reduced
Total Answered:

Levels of fibrin degradation products (FDPs) will be reduced


The activated partial thromboplastin time (APTT) will be below the normal range Feedback

The platelet count is likely to be elevated
Question Navigator

The patient has disseminated intravascular coagulation (DIC)


secondary to sepsis. Given the history, the most
Revision Notes
likely source is
the urinary tract.

Several coagulation abnormalities which may be seen in severe


sepsis include: Tags
APTT - elevated
PT - elevated
FDPs - elevated
D-dimers - elevated
Platelets - reduced
Fibrinogen - reduced
Protein C - reduced
Antithrombin - reduced.

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A 34-year-old female presented to the antenatal clinic at 24
weeks gestation complaining of general malaise,
Test Analysis
fatigue and
shortness of breath at rest. Her partner said that at times over
the past 24 hours she had been
vague and mildly confused. This was
her first pregnancy and had been uncomplicated. At her booking

appointment she had not reported any significant past medical


history. There was no abnormality detected at the
dating ultrasound
scan and her full blood count was within normal limits.

On examination her blood pressure was 122/80 mmHg; her booking


blood pressure had been 116/77 mmHg.
Her temperature was 37.8°C.
Heart sounds were normal. Chest was clear and abdominal examination
was
unremarkable. Examination of the peripheral and central nervous
system was unremarkable; but she was
disorientated in date, day and
time. She also seemed very agitated. There was a purpuric rash over
her shins.

She was admitted to the antenatal ward and a series of blood


tests sent. The results are shown below:

Haemoglobin 48 g/L (115 - 165)

Reticulocytes 16% (0.5 - 2.4)

White cell count 12.0 ×109/L (4 - 11)


Score:
Neutrophils 9.6 ×109/L (1.5 - 7.0)
Total Answered:
Lymphocytes 2.0 ×109/L (1.5 - 4.0)

Monocytes 0.2 ×109/L (0 - 0.8)


Question Navigator
Eosinophils 0.1 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)


Tags

Platelets 6.0 ×109/L (150 - 400)

Prothrombin time 16 s (11.5 - 15.5)

APTT 35 s (30 - 40)

Fibrinogen 5.6 g/L (1.8 - 5.4)

D-dimer <0.5 mg/L (<0.5)

Serum sodium 137 mmol/L (137 - 144)

Serum potassium 4.8 mmol/L (3.5 - 4.9)

Serum urea 8.0 mmol/L (2.5 - 7.5)

Serum creatinine 131 µmol/L (60 - 110)

Serum phosphate 1.38 mmol/L (0.8 - 1.4)

Serum corrected calcium 2.5 mmol/L (2.2 - 2.6)

Serum bilirubin 100 µmol/L (1 - 22)

Serum AST 30 U/L (1 - 31)

Serum alkaline phosphatase 120 U/L (45 - 105)

Serum lactate dehydrogenase 1898 U/L (10 - 250)

A peripheral blood film showed gross red cell fragmentation with


polychromasia with numerous spherocytes and
nucleated red
cells.

Urianalysis showed:

Blood +1

Protein +1

What is the correct management for this patient?

(Please select 1 option)



Delivery of baby as soon as possible


Intravenous broad-spectrum antibiotics


Observation on antenatal ward


Platelet infusion


Plasma exchange

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A 34-year-old female presented to the antenatal clinic at 24
weeks gestation complaining of general malaise,
Answer Statistics
fatigue and
shortness of breath at rest. Her partner said that at times over
the past 24 hours she had been
vague and mildly confused. This was
her first pregnancy and had been uncomplicated. At her booking

appointment she had not reported any significant past medical


history. There was no abnormality detected at the Test Analysis
dating ultrasound
scan and her full blood count was within normal limits.

On examination her blood pressure was 122/80 mmHg; her booking


blood pressure had been 116/77 mmHg.
Her temperature was 37.8°C.
Heart sounds were normal. Chest was clear and abdominal examination
was
unremarkable. Examination of the peripheral and central nervous
system was unremarkable; but she was
disorientated in date, day and
time. She also seemed very agitated. There was a purpuric rash over
her shins.

She was admitted to the antenatal ward and a series of blood


tests sent. The results are shown below:

Haemoglobin 48 g/L (115 - 165)

Reticulocytes 16% (0.5 - 2.4)

White cell count 12.0 ×109/L (4 - 11)

Neutrophils 9.6 ×109/L (1.5 - 7.0)

Lymphocytes 2.0 ×109/L (1.5 - 4.0)


Score:
Monocytes 0.2 ×109/L (0 - 0.8) Total Answered:
Eosinophils 0.1 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)


Feedback

Platelets 6.0 ×109/L (150 - 400)


Question Navigator
Prothrombin time 16 s (11.5 - 15.5)

APTT 35 s (30 - 40) Revision Notes


Fibrinogen 5.6 g/L (1.8 - 5.4)

D-dimer <0.5 mg/L (<0.5)


Tags
Serum sodium 137 mmol/L (137 - 144)

Serum potassium 4.8 mmol/L (3.5 - 4.9)

Serum urea 8.0 mmol/L (2.5 - 7.5)

Serum creatinine 131 µmol/L (60 - 110)

Serum phosphate 1.38 mmol/L (0.8 - 1.4)

Serum corrected calcium 2.5 mmol/L (2.2 - 2.6)

Serum bilirubin 100 µmol/L (1 - 22)

Serum AST 30 U/L (1 - 31)

Serum alkaline phosphatase 120 U/L (45 - 105)

Serum lactate dehydrogenase 1898 U/L (10 - 250)

A peripheral blood film showed gross red cell fragmentation with


polychromasia with numerous spherocytes and
nucleated red
cells.

Urianalysis showed:

Blood +1

Protein +1

What is the correct management for this patient?

(Please select 1 option)


Delivery of baby as soon as possible
Incorrect answer selected


Intravenous broad-spectrum antibiotics


Observation on antenatal ward


Platelet infusion

Plasma exchange
This is the correct answer

The differential diagnosis is a pregnancy associated thrombotic


microangiopathy, which includes:
Thrombotic thrombocytopenia purpura (TTP)
Haemolytic uraemic syndrome (HUS)
Pre-eclampsia
HELLP
Disseminated Intravascular coagulation (DIC)
Acute fatty liver of pregnancy
Systemic lupus erythematosus.

In this case the diagnosis favours TTP.

The full blood count shows there is a haemolytic anaemia-raised


reticulocyte count. The film shows
polychromasia and spherocytes in
keeping with haemolysis and fragmentation - suggesting a
microangiopathic
process. The LDH and bilirubin are also in keeping
with haemolysis. There is marked thrombocytopenia. The
coagulation
screen is only slightly abnormal; but importantly the fibrinogen is
not low and D-dimers normal
(excluding DIC). Renal function is
slightly impaired and liver enzymes really normal for
pregnancy.

The renal function is only slightly impaired and is against HUS


and pre-eclampsia. Her BP is normal and
urinalysis only slightly
off - again against pre-eclampsia.

The normal liver enzymes exclude HELLP and acute fatty liver of
pregnancy.

In TTP there is typically a pentad:


1. Microangiopathic haemolytic anaemia
2. Severe thrombocytopenia
3. Neurological involvement
4. Renal impairment
5. Fever.

However not all of these have to be present. Coagulation is


typically normal.

TTP in pregnancy typically occurs early - usually less than 24


weeks, whereas HELLP and pre-eclampsia occur
usually much later.
The only management option for TTP is plasma exchange. Delivery has
no effect on
disease.

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A 40-year-old gentleman presented to his general practitioner
complaining of tiredness, headaches and a pruritic
Test Analysis
rash after
bathing.

The symptoms of fatigue and headache had been present for


several months and at first he had attributed these
to stress from
work. He denied having any visual disturbance or focal limb
weakness. There was no history of
chest pain or breathlessness and
his bowel habit was normal. He had noticed the rash on his legs two
weeks
previously; it was the rash that had prompted him to consult
his GP.

He had no other past medical history of note. He had not smoked


for five years, but prior to that smoked for 20
years. He worked as
an investment banker, which he found stressful. He was not taking
any regular prescribed
medication, but had been taking a
homeopathic remedy for stress. He lived with his partner and their
5-year-old
son. There was a family history of hypertension.

On examination his face was plethoric. His blood pressure was


138/89 mmHg, with pulse 80 beats per minute
and regular. His heart
sounds were normal and all peripheral pulses were palpable. There
were no carotid
bruits. His chest was clear on auscultation with
normal vesicular breath sounds. His abdomen was soft and non-
tender
with a smooth mass extending two finger breadths below the left
costal margin.
Score:
The GP arranged a number of investigations, the results of which
are shown below:
Total Answered:
Haemoglobin 208 g/L (130-180)

Haematocrit 0.61 (0.4-0.52)


Question Navigator
White cell count 18.0 ×109/L (4-11)

Neutrophils 15.5 ×109/L (1.5-7) Tags

Lymphocytes 2.0 ×109/L (1.5-4.0)

Monocytes 0.2 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1)

Platelets 550 ×109/L (150-400)

Prothrombin time 19 s (11.5-15.5)

APTT 45 s (30-40)

Fibrinogen 4.0 g/L (1.8-5.4)

Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9)

Serum urea 7.5 mmol/L (2.5-7.5)

Serum creatinine 98 µmol/L (60-110)

Serum corrected calcium 2.3 mmol/L (2.2-2.6)

Serum phosphate 1.0 mmol/L (0.8-1.4)

Serum bilirubin 22 µmol/L (1-22)

Serum AST 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)


Serum ferritin 15 g/L (15-300)

On the basis of these results, the general practitioner referred


him to the general medicine outpatient clinic.

What is the likely diagnosis?

(Please select 1 option)



Apparent erythrocytosis due to the homeopathic medicine


Chronic myeloid leukaemia


Idiopathic erythrocytosis


Polycythaemia vera


Secondary erythrocytosis

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A 40-year-old gentleman presented to his general practitioner
complaining of tiredness, headaches and a pruritic
Answer Statistics
rash after
bathing.

The symptoms of fatigue and headache had been present for


several months and at first he had attributed these Test Analysis
to stress from
work. He denied having any visual disturbance or focal limb
weakness. There was no history of
chest pain or breathlessness and
his bowel habit was normal. He had noticed the rash on his legs two
weeks
previously; it was the rash that had prompted him to consult
his GP.

He had no other past medical history of note. He had not smoked


for five years, but prior to that smoked for 20
years. He worked as
an investment banker, which he found stressful. He was not taking
any regular prescribed
medication, but had been taking a
homeopathic remedy for stress. He lived with his partner and their
5-year-old
son. There was a family history of hypertension.

On examination his face was plethoric. His blood pressure was


138/89 mmHg, with pulse 80 beats per minute
and regular. His heart
sounds were normal and all peripheral pulses were palpable. There
were no carotid
bruits. His chest was clear on auscultation with
normal vesicular breath sounds. His abdomen was soft and non-
tender
with a smooth mass extending two finger breadths below the left
costal margin.

The GP arranged a number of investigations, the results of which


are shown below:

Haemoglobin 208 g/L (130-180) Score:


Haematocrit 0.61 (0.4-0.52) Total Answered:
White cell count 18.0 ×109/L (4-11)

Neutrophils 15.5 ×109/L (1.5-7) Feedback

Lymphocytes 2.0 ×109/L (1.5-4.0)


Question Navigator
Monocytes 0.2 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4) Revision Notes

Basophils 0.2 ×109/L (0-0.1)


Tags
Platelets 550 ×109/L (150-400)

Prothrombin time 19 s (11.5-15.5)

APTT 45 s (30-40)

Fibrinogen 4.0 g/L (1.8-5.4)

Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9)

Serum urea 7.5 mmol/L (2.5-7.5)

Serum creatinine 98 µmol/L (60-110)

Serum corrected calcium 2.3 mmol/L (2.2-2.6)

Serum phosphate 1.0 mmol/L (0.8-1.4)

Serum bilirubin 22 µmol/L (1-22)

Serum AST 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)


Serum ferritin 15 g/L (15-300)

On the basis of these results, the general practitioner referred


him to the general medicine outpatient clinic.

What is the likely diagnosis?

(Please select 1 option)


Apparent erythrocytosis due to the homeopathic medicine
Incorrect answer selected


Chronic myeloid leukaemia


Idiopathic erythrocytosis

Polycythaemia vera
This is the correct answer


Secondary erythrocytosis

There is a high haemoglobin (Hb) and haematocrit (Hct),


therefore there is polycythaemia. This is the most
significant
abnormality. There is also a mild neutrophilia and thrombocytosis
with a slightly raised basophil
count.

Absolute erythrocytosis, as opposed to apparent, is defined as


an Hct greater than 0.60 in males and Hct greater
than 0.56 in
females. We have therefore ruled out apparent erythrocytosis in
this man.

We are now left trying to decide if this is


1. Primary erythrocytosis (polycythaemia vera [PV])
2. Secondary erythrocytosis (due to some congenital or acquired
cause)
3. Idiopathic erythrocytosis (those with an absolute polycythaemia
but not primary and no secondary cause).

The diagnosis favours polycythaemia vera as opposed to secondary


or idiopathic for the following reasons:
Low serum ferritin is more common in PV than secondary.
High B12 is characteristic of PV due to
transcobalamin release from an increased granulocytic mass.
In the absence of liver disease, a palpable spleen is a
reliable sign of PV.
The pruritus is typically aquagenic as described here.

In PV, the white cell count and platelet count can be elevated.
In chronic myeloid leukaemia you may expect a
higher white cell
count and would not see the polycythaemia.

Also, note the prothrombin time and the activated partial


thromboplastin time (APTT) are prolonged. The reason
for this is
related to the ratio of plasma and citrate. In the blue tubes that
are used for coagulation tests the ratio
is normally 1 citrate to 9
of whole blood. If there is less plasma due to the polycythaemia
there will be excess
citrate and this will prolong coagulation
tests such as the APTT and prothrombin time.

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Work Smart MRCP Part 2 Written 2006 April

A 72-year-old woman attended the haematology day unit for


review.
Test Analysis
She complained of lethargy, headaches and visual disturbance
over the previous two weeks. Her daughter, who
accompanied her, had
noticed that her mother had been slightly confused in the past 24
hours.

On examination her blood pressure was 100/60 mmHg with a heart


rate of 88 beats per minute. She had some
mild pitting oedema of
her ankles and scattered bilateral basal fine crepitations. The
remainder of the
examination was normal. There was no evidence of
confusion and her mini mental score was 30.

Investigations revealed:

Haemoglobin 72 g/L (115-165)

MCV 88 fL (80-96)

White cell count 9.0 ×109/L (7-11)

Neutrophils 6.2 ×1099/L (1.5-7.0)

Lymphocytes 2.0 ×109/L (1.5-4.0)


Score:
Monocytes 0.6 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Question Navigator

Platelets 160 ×109/L (150-400)


Tags
 

Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.3-4.9)

Serum urea 9.0 mmol/L (2.5-7.5)

Serum creatinine 130 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (5-35)

Serum alkaline phosphatase 90 U/L (45-105)

Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.2 mmol/L (0.8-1.4)

Serum corrected calcium 2.6 mmol/L (2.2-2.6)

Serum albumin 26 g/L (37-49)

Serum total protein 120 g/L (61-76)

Serum C reactive protein 67 mg/L (<10)

Serum immunoglobulins

IgG 0.6 g/L (6.0-13.0)

IgM 0.2 g/L (0.4-2.5)

IgA 78 g/L (0.8-3.0)

IgE 10 g/L (<120)


Serum electrophoresis showed an IgA kappa monoclonal band with
immune paresis. The IgA kappa level was
76 g/L.

She was given three units of red cells, each unit running at an
infusion rate of two hours. Following the first unit
the patient
became acutely short of breath, confused and complained of a severe
headache. Her blood pressure
was recorded as 70/40 mmHg with a
regular heart rate of 120 beats per minute. She was afebrile with
oxygen
saturation of 88% on air.

What is the most likely cause of her acute deterioration?

(Please select 1 option)



Bacterial contamination of red cell unit


Transfusion associated graft verses host disease


Transfusion reaction


Transfusion related acute lung injury (TRALI)


Transfusion related fluid overload

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Work Smart MRCP Part 2 Written 2006 April

A 72-year-old woman attended the haematology day unit for


review.
Answer Statistics
She complained of lethargy, headaches and visual disturbance
over the previous two weeks. Her daughter, who
accompanied her, had
noticed that her mother had been slightly confused in the past 24
hours. Test Analysis
On examination her blood pressure was 100/60 mmHg with a heart
rate of 88 beats per minute. She had some
mild pitting oedema of
her ankles and scattered bilateral basal fine crepitations. The
remainder of the
examination was normal. There was no evidence of
confusion and her mini mental score was 30.

Investigations revealed:

Haemoglobin 72 g/L (115-165)

MCV 88 fL (80-96)

White cell count 9.0 ×109/L (7-11)

Neutrophils 6.2 ×1099/L (1.5-7.0)

Lymphocytes 2.0 ×109/L (1.5-4.0)

Monocytes 0.6 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4) Score:

Basophils 0.1 ×109/L (0-0.1) Total Answered:

Platelets 160 ×109/L (150-400)


Feedback
 

Serum sodium 137 mmol/L (137-144)


Question Navigator
Serum potassium 4.8 mmol/L (3.3-4.9)

Serum urea 9.0 mmol/L (2.5-7.5) Revision Notes


Serum creatinine 130 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (5-35) Tags


Serum alkaline phosphatase 90 U/L (45-105)

Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.2 mmol/L (0.8-1.4)

Serum corrected calcium 2.6 mmol/L (2.2-2.6)

Serum albumin 26 g/L (37-49)

Serum total protein 120 g/L (61-76)

Serum C reactive protein 67 mg/L (<10)

Serum immunoglobulins

IgG 0.6 g/L (6.0-13.0)

IgM 0.2 g/L (0.4-2.5)

IgA 78 g/L (0.8-3.0)

IgE 10 g/L (<120)


Serum electrophoresis showed an IgA kappa monoclonal band with
immune paresis. The IgA kappa level was
76 g/L.

She was given three units of red cells, each unit running at an
infusion rate of two hours. Following the first unit
the patient
became acutely short of breath, confused and complained of a severe
headache. Her blood pressure
was recorded as 70/40 mmHg with a
regular heart rate of 120 beats per minute. She was afebrile with
oxygen
saturation of 88% on air.

What is the most likely cause of her acute deterioration?

(Please select 1 option)


Bacterial contamination of red cell unit
Incorrect answer selected


Transfusion associated graft verses host disease


Transfusion reaction


Transfusion related acute lung injury (TRALI)

Transfusion related fluid overload


This is the correct answer

From blood results, it should be seen that she is anaemic with a


high level IgA, which is monoclonal.

Bacterial contamination of red cells is not very common, as most


pathogens do not survive the cold storage
conditions. Bacterial
contamination of platelet units is more common. Patients usually
present with fever, skin
flushing and rigors. Therefore not really
in keeping with the case above.

Transfusion associated graft versus host disease (TA-GVHD) is a


rare but usually fatal complication seen post
bone marrow
transplantation (BMT).

It is due to donor lymphocytes in transfused cellular


components, for example, blood, platelets. These donor
lymphocytes
recognise the recipient as foreign, and as the recipient is
immunocompromised due to the recent
BMT they cannot set up a
reaction to destroy these incoming lymphocytes.

In this case the recipient is the host and the incoming


lymphocytes are the graft.

The lymphocytes cause bone marrow failure, liver dysfunction and


gastrointestinal symptoms.

There is no cure, only preventative measures, in that all


cellular blood components given to BMT recipients need
to be
irradiated, this limits the functional activity of the
lymphocytes.

It usually occurs about 14 days after the affected transfusion.


In this case the FBC result is not in keeping at all
and the timing
is wrong.

With most types of transfusion reactions there is usually a


fever, this lady is afebrile.

Transfusion related acute lung injury is a severe acute reaction


characterised by respiratory distress, severe
hypoxia, fever and a
CXR showing perihilar and nodular shadowing in the mid and lower
zone, soon after
transfusion with no other apparent cause. In many
cases, preformed leucocyte antibodies have been found.

Cardiogenic causes of pulmonary oedema should be ruled out.

In contrast to patients with cardiogenic pulmonary oedema,


patients with TRALI have normal central venous
pressure and
normal/low pulmonary wedge pressure. Although TRALI is a
possibility here, the absence of a
fever again is not in keeping
and also the hypoxia is not as severe as one might expect.

Candidates should recognise that this lady has IgA myeloma from
the blood results. Although IgM, the largest
immunoglobulin and
nearly 100% intravascular, is most likely to cause hyperviscosity,
IgA and IgG3 tend to
aggregate and are more likely than other
isotypes or subclasses to be associated with hyperviscosity. This
lady
clearly has symptoms of hyperviscosity.

The plasma volume increases with increasing viscosity and may


compromise cardiac function as has happened
here even
pre-transfusion she is showing signs of cardiac compromise.
They should not be transfused until the viscosity has been
lowered as a rise in haematocrit can precipitate a
serious
worsening of their symptoms, which has occurred in this case.

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Work Smart MRCP Part 2 Written 2006 April

A 52-year-old lady was being treated for non-Hodgkins lymphoma.


She had tolerated the first three cycles of
Test Analysis
chemotherapy well, and
was attending for the fourth cycle. Over the course of several days
she complained of
an increasing tingling sensation in her hands and
feet that was also associated with a sensation of numbness.

She had a past history of hypertension for which she was taking
atenolol. Her other regular prescribed
medications comprised:
Omeprazole for a previous duodenal ulcer
Allopurinol as prophylaxis against tumour lysis
Ciprofloxacin and co-trimoxazole as antibacterial
prophylaxis
Itraconazole as anti-fungal prophylaxis.

There was no past history or family history of diabetes


mellitus.

Clinical examination revealed diminished sensation in a glove


and stocking distribution, consistent with
peripheral neuropathy.
Her blood pressure was 150/85 mmHg and a random blood glucose was
11 mmol/L.

Since she had received vincristine as part of the chemotherapeutic


regimen, this was considered to be the most
Score:
likely precipitating
cause.
Total Answered:

Which of the following drugs is responsible for possibly


exacerbating the vincristine neurotoxicity?

(Please select 1 option) Question Navigator



Allopurinol


Ciprofloxacin
Tags


Co-trimoxazole


Itraconazole


Omeprazole

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Work Smart MRCP Part 2 Written 2006 April

A 52-year-old lady was being treated for non-Hodgkins lymphoma.


She had tolerated the first three cycles of
Answer Statistics
chemotherapy well, and
was attending for the fourth cycle. Over the course of several days
she complained of
an increasing tingling sensation in her hands and
feet that was also associated with a sensation of numbness.
Test Analysis

She had a past history of hypertension for which she was taking
atenolol. Her other regular prescribed
medications comprised:
Omeprazole for a previous duodenal ulcer
Allopurinol as prophylaxis against tumour lysis
Ciprofloxacin and co-trimoxazole as antibacterial
prophylaxis
Itraconazole as anti-fungal prophylaxis.

There was no past history or family history of diabetes


mellitus.

Clinical examination revealed diminished sensation in a glove


and stocking distribution, consistent with
peripheral neuropathy.
Her blood pressure was 150/85 mmHg and a random blood glucose was
11 mmol/L.

Since she had received vincristine as part of the chemotherapeutic


regimen, this was considered to be the most
likely precipitating
cause.

Which of the following drugs is responsible for possibly


exacerbating the vincristine neurotoxicity? Score:

(Please select 1 option) Total Answered:

Allopurinol
Incorrect answer selected


Ciprofloxacin
Feedback


Co-trimoxazole
Question Navigator
Itraconazole
This is the correct answer


Omeprazole Revision Notes

Tags
Metabolism of vincristine is inhibited by itraconazole causing
increased risk of neurotoxicity which appears to be
dose-related.
The liver is the major excretory organ for vincristine in humans
and animals.

Neurological toxicity manifested as peripheral or autonomic


neuropathy is a feature of treatment with all vinca
alkaloids and
is a limiting side effect of vincristine.

If symptoms of neurotoxicity are severe, doses should be


reduced. Generally recovery of the nervous system is
very slow but
in most cases complete.

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A 33-year-old woman developed a fever (38.0°C) within 90 minutes
of starting a red cell transfusion three days
Test Analysis
post allogeneic bone
marrow transplant. Prior to the transfusion her temperature had
been 37.6°C.

On examination she appeared flushed, breathless and complained


of dizziness and a tight chest. Her heart rate
was 110 beats per
minute and regular, blood pressure was 90/60 mmHg and auscultation
of the chest revealed
widespread wheeze.

The staff nurse stopped the transfusion and checked a full blood
count, the result of which is shown below:

Haemoglobin 88 g/L (115-165)

White cell count 0.8 ×109/L (4-11)

Neutrophils 0.0 ×109/L (1.5-7)

Lymphocytes 0.5 ×109/L (1.5-4)

Monocytes 0.2 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4) Score:


Basophils 0.0 ×109/L (0-0.1) Total Answered:
Platelet count 12 ×109/L (150-400)
Question Navigator
What other action should be taken?

(Please select 1 option) Tags



Ask the blood bank for a further cross match of leucodepleted blood


Give paracetamol and keep close observation


Give paracetamol and restart transfusion at a slower rate


Give paracetamol, hydrocortisone and chlopheniramine


Start intravenous antibiotics to cover possible hospital acquired pneumonia

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Work Smart
A 33-year-old woman developed a fever (38.0°C) within 90 minutes
of starting a red cell transfusion three days
Answer Statistics
post allogeneic bone
marrow transplant. Prior to the transfusion her temperature had
been 37.6°C.

On examination she appeared flushed, breathless and complained


of dizziness and a tight chest. Her heart rate Test Analysis
was 110 beats per
minute and regular, blood pressure was 90/60 mmHg and auscultation
of the chest revealed
widespread wheeze.

The staff nurse stopped the transfusion and checked a full blood
count, the result of which is shown below:

Haemoglobin 88 g/L (115-165)

White cell count 0.8 ×109/L (4-11)

Neutrophils 0.0 ×109/L (1.5-7)

Lymphocytes 0.5 ×109/L (1.5-4)

Monocytes 0.2 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.0 ×109/L (0-0.1)


Score:
Platelet count 12 ×109/L (150-400)
Total Answered:
What other action should be taken?

(Please select 1 option) Feedback


Ask the blood bank for a further cross match of leucodepleted blood
Incorrect answer selected


Give paracetamol and keep close observation Question Navigator

Give paracetamol and restart transfusion at a slower rate
Revision Notes
Give paracetamol, hydrocortisone and chlopheniramine
This is the correct answer


Start intravenous antibiotics to cover possible hospital acquired pneumonia Tags

This is an immediate-type hypersensitivity reaction,


characterised by any combination of urticaria, erythema,

maculopapular rash, periorbital oedema, bronchospasm and


hypotension.

The appropriate treatment is stopping transfusion, colloids to


maintain BP, hydrocortisone, antihistamine,
paracetamol and
adrenaline where necessary.

Antibody causing the reaction is often unknown, but in some


cases it is directed against IgA (in recipients who
have become
sensitised).

All candidates should know that all red cell components in the
United Kingdom have been leucodepleted since
1999. This was
initially done because it was thought that this would reduce the
chances of Creutzfeldt-Jakob
disease transmission, as it was
initially thought to be transmitted in white blood cells; but it is
now known that it
is transmitted in plasma, red blood cells and
platelets - however all blood donations are now universally

leucodepleted.

This has reduced the number of febrile transfusion reactions,


which are due to anti-HLA antibodies in the
recipient serum or
granulocyte-specific antibodies. Answer 1 is not applicable.
Simply giving paracetamol is not going to help the allergic
nature of this reaction.

This patient may well have febrile neutropenia in addition to a


hypersensitivity reaction, and in practise, if she
was not already
on intravenous antibiotics you would start them at this time;
however the chest symptoms really
point to a hypersensitivity
reaction and these are the first priority in sorting out this
patient.

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A 55-year-old gentleman presented to the outpatient clinic after
being referred by his general practitioner.
Test Analysis
He complained of feeling lethargic and had lost 15 kg in weight.
He also complained of profuse sweating,
especially at night and
also had some upper abdominal discomfort. On examination, the
spleen was palpable 12
cm below the left costal margin.

Investigations revealed:

Haemoglobin 97 g/L (130-180)

White cell count 17.4 ×109/L (4-11)

Neutrophils 14.0 ×109/L (1.5-7)

Lymphocytes 1.5 ×109/L (1.5-4)

Monocytes 0.8 ×109/L (0-0.8)

Basophils 0.7 ×109/L (0-0.1)

Eosinohils 0.4 ×109/L (0.04-0.4) Score:

Platelets 550 ×109/L (150-400)


Total Answered:

The blood film was reported as follows: The neutrophils are left
shifted with numerous myelocytes present. There
Question Navigator
is an occasional
promyelocyte but no blasts. There are also a number of nucleated
red blood cells. There is a
thrombocytosis with platelet
anisocytosis.
Tags
What investigation should be performed next?

(Please select 1 option)



Immunophenotyping of peripheral blood


Lactate dehydrogenase (LDH) measurement


Molecular analysis of peripheral blood


Neutrophil alkaline phosphatase (NAP) score


Ultra sound scan (USS) of abdomen

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A 55-year-old gentleman presented to the outpatient clinic after
being referred by his general practitioner.
Answer Statistics
He complained of feeling lethargic and had lost 15 kg in weight.
He also complained of profuse sweating,
especially at night and
also had some upper abdominal discomfort. On examination, the
spleen was palpable 12 Test Analysis
cm below the left costal margin.

Investigations revealed:

Haemoglobin 97 g/L (130-180)

White cell count 17.4 ×109/L (4-11)

Neutrophils 14.0 ×109/L (1.5-7)

Lymphocytes 1.5 ×109/L (1.5-4)

Monocytes 0.8 ×109/L (0-0.8)

Basophils 0.7 ×109/L (0-0.1)

Eosinohils 0.4 ×109/L (0.04-0.4)

Platelets 550 ×109/L (150-400)


Score:
The blood film was reported as follows: The neutrophils are left
shifted with numerous myelocytes present. There Total Answered:
is an occasional
promyelocyte but no blasts. There are also a number of nucleated
red blood cells. There is a
thrombocytosis with platelet
anisocytosis.
Feedback
What investigation should be performed next?

(Please select 1 option) Question Navigator


Immunophenotyping of peripheral blood
Incorrect answer selected


Lactate dehydrogenase (LDH) measurement
Revision Notes

Molecular analysis of peripheral blood


This is the correct answer
Tags

Neutrophil alkaline phosphatase (NAP) score


Ultra sound scan (USS) of abdomen

The candidate should recognise anaemia, WBC and thrombocytosis


with a leucoerythroblastic blood film. The
patient has 'B'
symptoms - weight loss and night sweats with splenomegaly.

The differential is really between the myeloproliferative


disorders, chronic myeloid leukaemia (CML),
myelofibrosis and
essential thrombocythaemia (ET).

CML and myelofibrosis can be very difficult to distinguish in


the early stages on morphology alone or on clinical
findings and
the most useful and least invasive for the patient is molecular
analysis (PCR) of the peripheral
blood - most cases of CML are
usually associated with BCR-ABL translocation (t[9:22]), and this
can be
detected by PCR, whereas this is not seen in myelofibrosis
or ET.

If present it can usually be detected in the peripheral blood,


however in practice one would still eventually
proceed to a bone
marrow (BM) examination to assess morphology and you would still
also perform
conventional cytogenetics on the bone marrow (this is
done on a bone marrow sample rather than peripheral
blood because
the cellularity tends to be greater in the BM, giving lower failure
rates of the test).
LDH, NAP and USS are not going to help with making the
diagnosis, neither is immunophenotyping, as there are
no blasts in
the blood and this is not an acute leukaemia.

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Work Smart MRCP Part 2 Written July 2011

A 42-year-old man had received thrice weekly haemodialysis for


the previous 10 days via a subclavian line. Over
Test Analysis
the last two days
he developed problems in that the line had frequently been found to
be clotted. He had also
noticed some red skin lesions in the last
24 hours.

Four years previously (before starting dialysis) he had been


nephrotic and had developed a deep vein
thrombosis. This was
managed with low molecular weight heparin and then warfarin.

His medication included erythropoietin (EPO) and alphacalcidol.


Unfractionated heparin was being used for both
the subclavian and
dialysis lines.

On examination several necrotic skin lesions were noted on his


lower legs.

Further investigations revealed:

Haemoglobin 98 g/L (130-180)

White cell count 5.8 ×109/L (4-11)

Neutrophils 4.0 ×109/L (1.5-7)


Score:
Lymphocytes 0.9 ×109/L (1.5-4)
Total Answered:
Monocytes 0.4 ×109/L (0-0.8)

Eosinophils 0.4 ×109/L (0.04-0.4) Question Navigator


Basophils 0.1 ×109/L (0-0.1)
Tags
Platelet count 20 ×109/L (150-400)

Prothrombin time 16.0 s (11.5-15.5)

APTT 50 s (30-40)

Fibrinogen 7.6 g/L (1.8-5.4)

The blood film showed marked thrombocytopenia; the red cell


morphology was unremarkable.

What should be the next step in his management?

(Please select 1 option)



Prophylactic low molecular weight heparin


Remove subclavian line


Therapeutic lepirudin and stop heparin


Therapeutic low molecular weight heparin


Therapeutic unfractionated heparin

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Work Smart MRCP Part 2 Written July 2011

A 42-year-old man had received thrice weekly haemodialysis for


the previous 10 days via a subclavian line. Over
Answer Statistics
the last two days
he developed problems in that the line had frequently been found to
be clotted. He had also
noticed some red skin lesions in the last
24 hours.
Test Analysis
Four years previously (before starting dialysis) he had been
nephrotic and had developed a deep vein
thrombosis. This was
managed with low molecular weight heparin and then warfarin.

His medication included erythropoietin (EPO) and alphacalcidol.


Unfractionated heparin was being used for both
the subclavian and
dialysis lines.

On examination several necrotic skin lesions were noted on his


lower legs.

Further investigations revealed:

Haemoglobin 98 g/L (130-180)

White cell count 5.8 ×109/L (4-11)

Neutrophils 4.0 ×109/L (1.5-7)

Lymphocytes 0.9 ×109/L (1.5-4)

Monocytes 0.4 ×109/L (0-0.8) Score:

Eosinophils 0.4 ×109/L (0.04-0.4) Total Answered:

Basophils 0.1 ×109/L (0-0.1)


Feedback
Platelet count 20 ×109/L (150-400)

Prothrombin time 16.0 s (11.5-15.5)


Question Navigator
APTT 50 s (30-40)

Fibrinogen 7.6 g/L (1.8-5.4) Revision Notes


The blood film showed marked thrombocytopenia; the red cell
morphology was unremarkable.
Tags
What should be the next step in his management?

(Please select 1 option)


Prophylactic low molecular weight heparin
Incorrect answer selected


Remove subclavian line

Therapeutic lepirudin and stop heparin


This is the correct answer


Therapeutic low molecular weight heparin


Therapeutic unfractionated heparin

The most significant abnormality is his very low platelet count.


He also has anaemia, but this is common in renal
failure and the
fact that he is on EPO would suggest it is not new.

This is heparin induced thrombocytopenia (HIT). This is a


transient prothrombotic disorder that is initiated by
heparin. The
central features are thrombocytopenia and increased thrombin
production, the latter being a risk
factor for the development of
both arterial and venous thrombosis.
It is characterised by a more than 50% fall in platelet count or
platelet nadir 20-100 ×109/L that usually occurs
between
days five to 10 of starting heparin, but can occur earlier if
previously exposed.

There is often thrombosis or other sequelae (for example, skin


lesions) with no other cause for thrombocytopenia
evident.

The management of HIT is to stop heparin and full dose


anticoagulation with an alternative, such as lepirudin or

danaparoid commenced.

Warfarin should not be used until the platelet count has


recovered, that is, when the HIT process has finished.

When introduced, an alternative anticoagulant must be continued


until the international normalised ratio (INR) is
therapeutic for
two consecutive days. This is because when warfarin is introduced,
the protein C level falls first
before the other clotting factors,
therefore there is a prothrombotic period before anticoagulation is
achieved with
warfarin.

Further recent guidelines on HIT can be found at the website of


the British
Society for Haematology.

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Work Smart MRCP Part 2 Written July 2011

A 49-year-old female attended the ward for pre-clerking for a


routine laparoscopic cholecystectomy.
Test Analysis
Her past medical history included surgery for an injury to her
left arm one year previously. Her only medication
was hormone
replacement therapy. Apart from occasional episodes of right upper
quadrant pain secondary to
gall stones, she was otherwise very
well.

Routine pre-operative bloods were performed and the results are


shown below:

Haemoglobin 138 g/L (115 - 165)

White cell count 7.9 ×109/L (4 - 11)

Neutrophils 5.0 ×109/L (1.5 - 7)

Lymphocytes 2.0 ×109/L (1.5 - 4)

Monocytes 0.4 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1) Score:

Platelet count 220 ×109/L (150 - 400)


Total Answered:

Prothrombin time 13.0 s (11.5 - 15.5)


Question Navigator
APTT 79 s (30 - 40)

Fibrinogen 4.6 g/L (1.8 - 5.4)


Tags
APTT (50:50 mix with normal plasma) 70 s

The laparoscopic cholecystectomy was performed without


complications.

What is the most likely explanation for the laboratory


results?

(Please select 1 option)



Factor VIII deficiency


Factor VIII inhibitor


Factor IX deficiency


Lupus anticoagulant present


von Willebrand disease

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Work Smart MRCP Part 2 Written July 2011

A 49-year-old female attended the ward for pre-clerking for a


routine laparoscopic cholecystectomy.
Answer Statistics
Her past medical history included surgery for an injury to her
left arm one year previously. Her only medication
was hormone
replacement therapy. Apart from occasional episodes of right upper
quadrant pain secondary to Test Analysis
gall stones, she was otherwise very
well.

Routine pre-operative bloods were performed and the results are


shown below:

Haemoglobin 138 g/L (115 - 165)

White cell count 7.9 ×109/L (4 - 11)

Neutrophils 5.0 ×109/L (1.5 - 7)

Lymphocytes 2.0 ×109/L (1.5 - 4)

Monocytes 0.4 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)

Platelet count 220 ×109/L (150 - 400)


Score:
Prothrombin time 13.0 s (11.5 - 15.5)
Total Answered:
APTT 79 s (30 - 40)

Fibrinogen 4.6 g/L (1.8 - 5.4)


Feedback
APTT (50:50 mix with normal plasma) 70 s

The laparoscopic cholecystectomy was performed without


complications. Question Navigator

What is the most likely explanation for the laboratory


results?
Revision Notes
(Please select 1 option)
Factor VIII deficiency
Incorrect answer selected Tags

Factor VIII inhibitor


Factor IX deficiency

Lupus anticoagulant present


This is the correct answer


von Willebrand disease

The candidate should recognise that the only abnormality on the


blood results is a significantly prolonged
activated partial
thromboplastin time (APTT), which does not correct by a significant
amount when patient's
plasma is mixed with normal plasma.

If this were a factor deficiency then greater than 50%


correction would be expected. This is therefore in keeping
with an
inhibitor of some kind.

Factor VIII deficiency, factor IX deficiency and von Willebrand


disease are therefore ruled out. The clinical details
and history
are against these too - female, previous surgery, uncomplicated,
etc.

This leaves us with either factor VIII inhibitor or the presence


of lupus anticoagulant.
The development of a factor VIII inhibitor is usually associated
with some bleeding symptoms, and in fact factor
inhibitors are
usually associated with a high degree of morbidity and mortality.
This patient is very well, with no
bleeding symptoms at all.

In addition, factor VIII inhibitors are usually time dependent.


As a result when the initial 50:50 mix is done there is
correction
of the APTT; but if you repeat the APTT after allowing the 50:50
mix to incubate for two hours, there
will be no correction.

The most likely answer is therefore that the lupus anticoagulant


(LAC) is present.

Coagulation tests to demonstrate the presence of the LAC are as


follows:
Prolongation of a phospholipid-dependent coagulation test, for
example, APTT, kaolin clotting time or
others.
Demonstration of inhibitor by failing to correct the above
coagulation test on 50:50 mixing studies by
more than 50%.
Demonstrate phospholipid dependence-correction of the
coagulation test used in (1) with phospholipid.

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A 42-year-old gentleman presented to the Emergency department
with a 24 hour history of lower abdominal
Test Analysis
pain. He had seen his
general practitioner five days previously for cramps in his legs at
night. The GP had taken
a full blood count and prescribed him
quinine sulphate, he had taken just one dose the following
evening.

His medical history was unremarkable, although he had a previous


hospital admission with similar abdominal
pain in his teens. No
cause was found for that episode.

On examination he appeared jaundiced. His blood pressure was


92/60 mmHg, but no other abnormality was
detected.

Further investigations revealed:

Blood count five days previously (arranged by GP):

Haemoglobin 140 g/L (130 - 180)

White cell count 8.7 ×109/L (4 - 11)

Neutrophils 6.0 ×109/L (1.5 - 7)


Score:
Lymphocytes 1.7 ×109/L (1.5 - 4)
Total Answered:
Monocytes 0.6 ×109/L (0 - 0.8)

Eosinophils 0.3 ×109/L (0.04 - 0.4) Question Navigator


Basophils 0.1 ×109/L (0 - 0.1)
Tags
Platelet count 195 ×109/L (150 - 400)

Full blood count on admission to hospital:

Haemoglobin 98 g/L (130 - 180)

White cell count 15..5 ×109/L (4 - 11)

Neutrophils 10.0 ×109/L (1.5 - 7)

Lymphocytes 4.0 ×109/L (1.5 - 4)

Monocytes 1.0 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)

Platelet count 466 ×109/L (150 - 400)

Reticulocyte count 10% -

Direct Coombs test: Negative -

Blood Film: Red cell anisocytosis, spherocytes and irregularly


contracted red cells. Heinz bodies inclusions are
present.

Serum sodium 137 mmol/L (137 - 144)

Serum potassium 4.8 mmol/L (3.5 - 4.9)

Serum urea 7.0 mmol/L (2.5 - 7.5)


Serum creatinine 100 mol/L (60 - 110)

Serum aspartate aminotransferase 30 U/L (1 - 31)

Serum alkaline phosphatase 90 U/L (45 - 105)

Serum total bilirubin 122 mol/L (1 - 22)

Serum phosphate 1.0 mmol/L (0.8 - 1.4)

Serum corrected calcium 2.1 mmol/L (2.2 - 2.6)

Serum albumin 37 g/L (37 - 49)

Serum total protein 70 g/L (61 - 76)

Serum C reactive protein 40 mg/L -

What is the most likely diagnosis ?

(Please select 1 option)



Autoimmune haemolytic anaemia


Glucose-6-phosphate dehydrogenase deficiency


Hereditary spherocytosis


Obstructive jaundice


Paroxysmal nocturnal haemoglobinuria

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Work Smart
A 42-year-old gentleman presented to the Emergency department
with a 24 hour history of lower abdominal
Answer Statistics
pain. He had seen his
general practitioner five days previously for cramps in his legs at
night. The GP had taken
a full blood count and prescribed him
quinine sulphate, he had taken just one dose the following
evening.
Test Analysis
His medical history was unremarkable, although he had a previous
hospital admission with similar abdominal
pain in his teens. No
cause was found for that episode.

On examination he appeared jaundiced. His blood pressure was


92/60 mmHg, but no other abnormality was
detected.

Further investigations revealed:

Blood count five days previously (arranged by GP):

Haemoglobin 140 g/L (130 - 180)

White cell count 8.7 ×109/L (4 - 11)

Neutrophils 6.0 ×109/L (1.5 - 7)

Lymphocytes 1.7 ×109/L (1.5 - 4)

Monocytes 0.6 ×109/L (0 - 0.8) Score:

Eosinophils 0.3 ×109/L (0.04 - 0.4) Total Answered:

Basophils 0.1 ×109/L (0 - 0.1)


Feedback
Platelet count 195 ×109/L (150 - 400)

Full blood count on admission to hospital: Question Navigator

Haemoglobin 98 g/L (130 - 180)


Revision Notes
White cell count 15..5 ×109/L (4 - 11)

Neutrophils 10.0 ×109/L (1.5 - 7) Tags


Lymphocytes 4.0 ×109/L (1.5 - 4)

Monocytes 1.0 ×109/L (0 - 0.8)

Eosinophils 0.4 ×109/L (0.04 - 0.4)

Basophils 0.1 ×109/L (0 - 0.1)

Platelet count 466 ×109/L (150 - 400)

Reticulocyte count 10% -

Direct Coombs test: Negative -

Blood Film: Red cell anisocytosis, spherocytes and irregularly


contracted red cells. Heinz bodies inclusions are
present.

Serum sodium 137 mmol/L (137 - 144)

Serum potassium 4.8 mmol/L (3.5 - 4.9)

Serum urea 7.0 mmol/L (2.5 - 7.5)


Serum creatinine 100 mol/L (60 - 110)

Serum aspartate aminotransferase 30 U/L (1 - 31)

Serum alkaline phosphatase 90 U/L (45 - 105)

Serum total bilirubin 122 mol/L (1 - 22)

Serum phosphate 1.0 mmol/L (0.8 - 1.4)

Serum corrected calcium 2.1 mmol/L (2.2 - 2.6)

Serum albumin 37 g/L (37 - 49)

Serum total protein 70 g/L (61 - 76)

Serum C reactive protein 40 mg/L -

What is the most likely diagnosis ?

(Please select 1 option)


Autoimmune haemolytic anaemia
Incorrect answer selected

Glucose-6-phosphate dehydrogenase deficiency


This is the correct answer


Hereditary spherocytosis


Obstructive jaundice


Paroxysmal nocturnal haemoglobinuria

The important point is that five days previously the FBC was
normal. Therefore in the steady state, as it were,
this man has
normal parameters. The other thing to recognise is that this is
haemolysis - low Hb, raised
reticulocytes and raised bilirubin.
Obstructive jaundice is therefore easy to rule out.

The DCT is negative - ruling out immune haemolysis.

In hereditary spherocytosis the majority of patients have low Hb


due to continued red cell destruction, although
few can have a
chronic compensated haemolysis with normal Hb and reticulocyte
count. The fact that his FBC
was normal five days ago tends to go
against HS.

We are really left with paroxysmal nocturnal haemoglobinuria and


G6PD deficiency. The FBC results would fit
with either. What
clinches the diagnosis for the latter is the information regarding
quinine, the irregularly
contracted red cells and Heinz bodies on
film.

G6PD is an X linked disorder, red cells loose their ability to


generate NADPH from NADP and as a result,
oxidised glutathione can
not be reduced. This is essential in maintaining the integrity of
the red cell membrane,
and so without it the cells are prone to
oxidative stress and hence haemolysis.

The majority of the time individuals are unaffected by this, it


is only when some additional stress is incurred that
the supply of
NADPH becomes inadequate and the red cell is susceptible to
oxidative stress and undergoes
haemolysis. Quinine is a classic
drug that can lead to this additional stress, and haemolysis occurs
one to three
days after ingestion. The classic finding on the film
is irregularly contracted red cells (bite cells) and Heinz

bodies.

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Work Smart MRCP Part 2 Written July 2011

A 44-year-old woman was admitted to the gynaecology ward for


investigation of menorrhagia. After receiving the
Test Analysis
results of her
initial investigations the gynaecology team request a medical
review.

She was known to be anaemic and had been prescribed ferrous


sulphate by her GP. However, she experienced
severe abdominal pain
and constipation when taking oral iron supplements and had
discontinued the tablets of
her own accord.

In addition to menorrhagia, she complained of constant fatigue


and also experienced breathlessness on exertion
that was limiting
her daily activities.

Investigations revealed:

Haemoglobin 68 g/L (115-165)

MCV 74 fL (80-96)

MCH 26 pg (28-32)

White cell count 7.3 ×109/L (4-11)

Neutrophils 3.0 ×109/L (1.5-7) Score:

Lymphocytes 3.0 ×109/L (1.5-4) Total Answered:

Monocytes 0.8 ×109/L (0-0.8)


Question Navigator
Eosinophils 0.4 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Tags

Platelet count 566 ×109/L (150-400)

Serum ferritin 5 g/L (15-300)

She has already had oral iron at a sub-optimal therapeutic dose,


owing to side effects, for four weeks.

What should be the next step in this patient's


management?

(Please select 1 option)



Blood transfusion


Consider stopping iron replacement until haemoglobin
electrophoresis is done, as this is more likely to be a

haemoglobinopathy


Continue with the current oral iron replacement


Intramuscular iron replacement


Intravenous iron replacement

Submit answer
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Work Smart MRCP Part 2 Written July 2011

A 44-year-old woman was admitted to the gynaecology ward for


investigation of menorrhagia. After receiving the
Answer Statistics
results of her
initial investigations the gynaecology team request a medical
review.

She was known to be anaemic and had been prescribed ferrous


sulphate by her GP. However, she experienced Test Analysis
severe abdominal pain
and constipation when taking oral iron supplements and had
discontinued the tablets of
her own accord.

In addition to menorrhagia, she complained of constant fatigue


and also experienced breathlessness on exertion
that was limiting
her daily activities.

Investigations revealed:

Haemoglobin 68 g/L (115-165)

MCV 74 fL (80-96)

MCH 26 pg (28-32)

White cell count 7.3 ×109/L (4-11)

Neutrophils 3.0 ×109/L (1.5-7)

Lymphocytes 3.0 ×109/L (1.5-4)


Score:
Monocytes 0.8 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.4 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Feedback

Platelet count 566 ×109/L (150-400)


Question Navigator
Serum ferritin 5 g/L (15-300)

She has already had oral iron at a sub-optimal therapeutic dose,


owing to side effects, for four weeks. Revision Notes
What should be the next step in this patient's
management?
Tags
(Please select 1 option)
Blood transfusion
Incorrect answer selected


Consider stopping iron replacement until haemoglobin
electrophoresis is done, as this is more likely to be a

haemoglobinopathy


Continue with the current oral iron replacement


Intramuscular iron replacement

Intravenous iron replacement


This is the correct answer

There is obviously iron deficiency:


Low haemoglobin (Hb)
Low mean corpuscular volume (MCV) and mean cell haemoglobin
(MCH) and
Very low ferritin.

Sometimes a haemoglobinopathy can co-exist; but at 44-years-old


it would be a minor haemoglobinopathy, that
is, she would not have
got to this age with a major state and not know about it. So before
electrophoresis can be
interpreted it would be necessary to correct
any iron deficiency as it can affect HbA2 level.
Clearly, continuing with oral iron replacement is not great for
this lady. She is experiencing side effects which are
limiting her
compliance, which is very often the case. Her Hb remains 6.8 and it
will probably take months and
months to increase her Hb.

Intramuscular iron is no longer recommended due to side effects,


mainly allergic type reactions.

Intravenous iron replacement is now very widely used in this


situation. Although it carries some risk of allergic
side effects
and needs IV access, it is effective and very well tolerated.

A blood transfusion is not the answer here, she needs her stores
of iron to be replenished properly, and of
course a blood
transfusion should not be given unless absolutely necessary in any
situation, because of all the
potential problems it comes with.

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Work Smart MRCP Part 2 Written July 2011

A 62-year-old gentleman was followed up every six months in the


haematology clinic.
Test Analysis
During his most recent appointment he complained of a headache,
blurred vision, aching bones, and extreme
lethargy. His wife
reported that he has been very vague at times over the previous
four weeks and drowsy when
he was awake.

On examination he appeared pale with blood pressure 125/65 mmHg.


He was afebrile. Respiratory rate was 12
breaths per minute.
Cardiovascular, chest and abdominal examination were
unremarkable.

Further investigations showed:

Haemoglobin 82 g/L (130-180)

MCV 88 fL (80-96)

White cell count 9.0 ×109/L (4-11)

Neutrophils 6.2 ×109/L (1.5-7)

Lymphocytes 2.0 ×109/L (1.5-4)


Score:
Monocytes 0.6 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Question Navigator

Platelet count 160 ×109/L (150-400)


Tags
Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9)

Serum urea 9.0 mmol/L (2.5-7.5)

Serum creatinine 130 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)

Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.2 mmol/L (0.8-1.4)

Serum corrected calcium 2.7 mmol/L (2.2-2.6)

Serum albumin 26 g/L (37-49)

Serum total protein 120 g/L (61-76)

Serum C-reactive protein 67 mg/L -

Serum immunoglobulins:

IgG 0.6 g/L (6.0-13.0)

IgA 78 g/L (0.8-3.0)

IgM 0.2 g/L (0.4-2.5)

Serum electrophoresis showed a monoclonal band (IgA kappa) with


immune paresis. The IgA kappa level was
76 g/L.
Which of the following would be the best treatment option for
this gentleman?

(Please select 1 option)



Blood transfusion


Chemotherapy


Intravenous fluids and furosemide


Plasma exchange


Plasma exchange and chemotherapy

Submit answer
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Work Smart MRCP Part 2 Written July 2011

A 62-year-old gentleman was followed up every six months in the


haematology clinic.
Answer Statistics
During his most recent appointment he complained of a headache,
blurred vision, aching bones, and extreme
lethargy. His wife
reported that he has been very vague at times over the previous
four weeks and drowsy when Test Analysis
he was awake.

On examination he appeared pale with blood pressure 125/65 mmHg.


He was afebrile. Respiratory rate was 12
breaths per minute.
Cardiovascular, chest and abdominal examination were
unremarkable.

Further investigations showed:

Haemoglobin 82 g/L (130-180)

MCV 88 fL (80-96)

White cell count 9.0 ×109/L (4-11)

Neutrophils 6.2 ×109/L (1.5-7)

Lymphocytes 2.0 ×109/L (1.5-4)

Monocytes 0.6 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4) Score:

Basophils 0.1 ×109/L (0-0.1) Total Answered:

Platelet count 160 ×109/L (150-400)


Feedback
Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9) Question Navigator


Serum urea 9.0 mmol/L (2.5-7.5)

Serum creatinine 130 µmol/L (60-110) Revision Notes


Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)


Tags

Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.2 mmol/L (0.8-1.4)

Serum corrected calcium 2.7 mmol/L (2.2-2.6)

Serum albumin 26 g/L (37-49)

Serum total protein 120 g/L (61-76)

Serum C-reactive protein 67 mg/L -

Serum immunoglobulins:

IgG 0.6 g/L (6.0-13.0)

IgA 78 g/L (0.8-3.0)

IgM 0.2 g/L (0.4-2.5)

Serum electrophoresis showed a monoclonal band (IgA kappa) with


immune paresis. The IgA kappa level was
76 g/L.
Which of the following would be the best treatment option for
this gentleman?

(Please select 1 option)


Blood transfusion
Incorrect answer selected


Chemotherapy


Intravenous fluids and furosemide


Plasma exchange

Plasma exchange and chemotherapy


This is the correct answer

The patient has a blood picture and symptoms (bone pain) in


keeping with multiple myeloma.

It is likely that the patient has had a paraprotein for some


time, maybe a monoclonal gammopathy of
undetermined significance
(MGUS) as it was detected under six monthly follow up.

He now has very high-level IgA kappa, with bony pain, anaemia,
impaired renal function and high calcium. He
also has quite marked
symptoms of hyperviscosity secondary to the paraprotein.

Although IgM, the largest immunoglobulin and nearly 100%


intravascular, is most likely to cause hyperviscosity,
IgA and IgG3
tend to aggregate and are more likely than other isotypes or
subclasses to be associated with
hyperviscosity.

Treating the anaemia with a transfusion at this stage can


exacerbate the hyperviscosity.

The calcium is not the cause of his main problems now and so
treating this with IV fluids and furosemide is not a
priority.

Patients with myeloma with high paraprotein levels and symptoms


related to hyperviscosity should have urgent
plasma exchange,
chemotherapy needs to then be instituted promptly to control the
disease process and
prevent symptoms reoccurring. If transfusion is
absolutely necessary then an exchange transfusion should be

done.

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Work Smart MRCP Part 2 Written July 2011

A 17-year-old man was being investigated for a long history of


malaise.
Test Analysis
His past medical history included recurrent episodes of anaemia
but the cause had never been established.
There was no past history
or family history of note and apart from the malaise he reported no
other symptoms.

On examination he was mildly jaundiced. He had a blood pressure


of 120/75 mmHg and his pulse was 80 beats
per minute. His spleen
was palpable 6 cm below the left costal margin. No other
abnormality was found.

Results of investigations are shown below:

Haemoglobin 84 g/L (130-180)

MCV 76 fL (80-96)

MCH 29 pg (28-32)

MCHC 40 g/dL (32-35)

White cell count 11.0 ×109/L (4-11)

Neutrophils 7.0 ×109/L (1.5-7)


Score:
Lymphocytes 3.2 ×109/L (1.5-4)
Total Answered:
Monocytes 0.5 ×109/L (0-0.8)

Eosinophils 0.2 ×109/L (0.04-0.4) Question Navigator

Basophils 0.1 ×109/L (0-0.1)


Tags
Platelets 366 ×109/L (150-400)

Reticulocyte count 9.0%

Serum ferritin 45 g/L (15-300)

Serum folate 1.2 g/L (2.0-11.0)

Direct Coombs test Negative

Osmotic fragility test Increased osmotic fragility

Serum sodium 139 mmol/L (137-144)

Serum potassium 4.5 mmol/L (3.5-4.9)

Serum urea 4.5 mmol/L (2.5-7.5)

Serum creatinine 60 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 56 U/L (45-105)

Serum total bilirubin 102 µmol/L (1-22)

Serum phosphate 0.9 mmol/L (0.8-1.4)

Serum corrected calcium 2.2 mmol/L (2.2-2.6)

Serum albumin 40 g/L (37-49)

Serum total protein 65 g/L (61-76)

The blood film showed red cell poikilocytes with spherocytes and
some polychromasia.
What is the diagnosis?

(Please select 1 option)



Aplastic anaemia


Autoimmune haemolysis


Hereditary elliptocytosis


Hereditary spherocytosis


Paroxysmal cold haemoglobinuria

Submit answer
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Work Smart MRCP Part 2 Written July 2011

A 17-year-old man was being investigated for a long history of


malaise.
Answer Statistics
His past medical history included recurrent episodes of anaemia
but the cause had never been established.
There was no past history
or family history of note and apart from the malaise he reported no
other symptoms. Test Analysis
On examination he was mildly jaundiced. He had a blood pressure
of 120/75 mmHg and his pulse was 80 beats
per minute. His spleen
was palpable 6 cm below the left costal margin. No other
abnormality was found.

Results of investigations are shown below:

Haemoglobin 84 g/L (130-180)

MCV 76 fL (80-96)

MCH 29 pg (28-32)

MCHC 40 g/dL (32-35)

White cell count 11.0 ×109/L (4-11)

Neutrophils 7.0 ×109/L (1.5-7)

Lymphocytes 3.2 ×109/L (1.5-4)

Monocytes 0.5 ×109/L (0-0.8) Score:

Eosinophils 0.2 ×109/L (0.04-0.4)


Total Answered:

Basophils 0.1 ×109/L (0-0.1)


Feedback
Platelets 366 ×109/L (150-400)

Reticulocyte count 9.0% Question Navigator


Serum ferritin 45 g/L (15-300)

Serum folate 1.2 g/L (2.0-11.0)


Revision Notes

Direct Coombs test Negative


Tags
Osmotic fragility test Increased osmotic fragility

Serum sodium 139 mmol/L (137-144)

Serum potassium 4.5 mmol/L (3.5-4.9)

Serum urea 4.5 mmol/L (2.5-7.5)

Serum creatinine 60 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 56 U/L (45-105)

Serum total bilirubin 102 µmol/L (1-22)

Serum phosphate 0.9 mmol/L (0.8-1.4)

Serum corrected calcium 2.2 mmol/L (2.2-2.6)

Serum albumin 40 g/L (37-49)

Serum total protein 65 g/L (61-76)

The blood film showed red cell poikilocytes with spherocytes and
some polychromasia.
What is the diagnosis?

(Please select 1 option)


Aplastic anaemia
Incorrect answer selected


Autoimmune haemolysis


Hereditary elliptocytosis

Hereditary spherocytosis
This is the correct answer


Paroxysmal cold haemoglobinuria

Candidates should recognise that there is anaemia with a low


mean corpuscular volume (MCV) but high mean
cell haemoglobin
concentration (MCHC); the latter suggests hyperdense cells.

There is a haemolytic process, as the reticulocyte count and


bilirubin are high, there is, as a result, folate
depletion. The
film shows polychromasia (suggesting increased red cell turnover)
with red cell shape changes
(anisocytosis) and spherocytes - again
this is suggestive of haemolysis.

The direct Coombs' test is negative. This is therefore not


an immune haemolysis.

In aplastic anaemia there is absence of a reticulocytosis - the


red cell precursors are simply not present.

In hereditary elliptocytosis, one would expect to find


elliptocytes on the blood film.

The classic clue that this is hereditary spherocytosis (HS) is


the spherocytes, the high MCHC, indicating
hyperdense cells, and
the increased osmotic fragility of the red cells.

HS is the commonest cause of inherited chronic haemolysis in


Northern Europe and North America. The
abnormal morphology and
shorter life span of the red blood cells are attributable to a
deficiency or dysfunction
of one of the constituents of the red
cell cytoskeleton.

Diagnosis is usually made in childhood or young adult life, when


there is anaemia and splenomegaly. Diagnosis
is usually made on
history, positive family history and laboratory data. Further
confirmatory tests are sometimes
required. This used to be the
osmotic fragility test; but this has now been replaced by the
eosin-5-maleimide
binding to red cells and then being detected by
flow cytometry.

Treatment is with splenectomy, if haemolysis is severe


enough.

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A 23-year-old woman was brought to the Emergency department. She
was known to have acute myeloid
Test Analysis
leukaemia and was undergoing
chemotherapy.

She had been unwell for the last eight hours and described
having shaking shivers and sweats at home. She
reported feeling
very nauseated and had had some diarrhoea. She had no other
symptoms.

On examination her temperature was 39.4°C. Her blood pressure


was 90/55 mmHg with a heart rate of 136
beats per minute and
regular. She had some oral candidiasis and a Hickman line was in
situ. Cardiovascular,
chest and abdominal examination were
unremarkable.

Investigations showed:

Haemoglobin 84 g/L (115-165)

MCV 97 fL (80-96)

MCH 30 pg (28-32)

White cell count 0.1 ×109/L (4-11)

Neutrophils 0.0 ×109/L (1.5-7) Score:

Lymphocytes 0.0 ×109/L (1.5-4) Total Answered:

Monocytes 0.1 ×109/L (0-0.8)


Question Navigator
Eosinophils 0.0 ×109/L (0.04-0.4)

Basophils 0.0 ×109/L (0-0.1) Tags

Platelet count 6 ×109/L (150-400)

Her chest x ray was normal.

How would you manage this patient?

(Please select 1 option)



Await blood culture results before any specific therapy


Remove Hickman line, as most likely cause of problems


Start antipseudomonal agent combined with aminoglycoside


Start ciprofloxacin


Start systemic antifungal therapy

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Work Smart
A 23-year-old woman was brought to the Emergency department. She
was known to have acute myeloid
Answer Statistics
leukaemia and was undergoing
chemotherapy.

She had been unwell for the last eight hours and described
having shaking shivers and sweats at home. She Test Analysis
reported feeling
very nauseated and had had some diarrhoea. She had no other
symptoms.

On examination her temperature was 39.4°C. Her blood pressure


was 90/55 mmHg with a heart rate of 136
beats per minute and
regular. She had some oral candidiasis and a Hickman line was in
situ. Cardiovascular,
chest and abdominal examination were
unremarkable.

Investigations showed:

Haemoglobin 84 g/L (115-165)

MCV 97 fL (80-96)

MCH 30 pg (28-32)

White cell count 0.1 ×109/L (4-11)

Neutrophils 0.0 ×109/L (1.5-7)

Lymphocytes 0.0 ×109/L (1.5-4)


Score:
Monocytes 0.1 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.0 ×109/L (0.04-0.4)

Basophils 0.0 ×109/L (0-0.1) Feedback

Platelet count 6 ×109/L (150-400)


Question Navigator
Her chest x ray was normal.

How would you manage this patient? Revision Notes

(Please select 1 option)


Tags
Await blood culture results before any specific therapy
Incorrect answer selected


Remove Hickman line, as most likely cause of problems

Start antipseudomonal agent combined with aminoglycoside


This is the correct answer


Start ciprofloxacin


Start systemic antifungal therapy

This is dealing with febrile neutropenic sepsis.

The patient is pancytopenic post-chemotherapy and is clearly


septic. There are no specific symptoms suggestive
of focus of
infection. This is an emergency, and the patient needs to start
antibacterial treatment ASAP.

Early death is usually due to Gram negative sepsis; later


mortality is from other bacteria, fungal infection or viral

infection.

Although in recent years there has been a steady shift towards


Gram positive sepsis, Gram negative organisms
remain the most
common. It is therefore necessary to treat with a third or fourth
generation antipseudomonal
agent, for example
piperacillin/tazobactam, and at present most units will still use
an aminoglycoside agent -
although recent evidence suggests that
monotherapy alone with a β lactam is sufficient.

Although the Hickman line is a potential source, at this stage


removing it is not the initial priority. It may need to
be removed
if cultures indicate or if temperature fails to settle.

Ciprofloxacin will not give enough spectrum of antibacterial


activity.

Systemic antifungal therapy is not first line management in such


cases; but would be introduced if after seven
days of
antibacterials the fever had still not settled.

Clearly, awaiting blood culture results is not an option.

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A 30-year-old man was referred to outpatients because of
abnormal liver function tests.
Test Analysis
He had attended his general practitioner complaining of lethargy
and feeling generally unwell. He worked at his
family's dairy
farm. He drank alcohol regularly, consuming on average 30 units per
week. He took thyroxine for
hypothyroidism. He denied drug abuse;
he had never had a blood transfusion and had never travelled
abroad.

On examination he looked generally well, he was not overtly


jaundiced, there were no signs of chronic liver
disease.

His tests revealed

Haemoglobin 142 g/L (130-180)

White cells 9.0 ×109/L (4-11)

Platelets 300 ×109/L (150-400)

MCV 102 fL (80-96)

Albumin 40 g/L (37-49)


Score:
Bilirubin 35 μmol/L (1-22)
Total Answered:
Alanine transferase 210 U/L (5-35)

Alkaline phosphatase 140 U/L (45-105)


Question Navigator
Gamma gluteryltransferase 120 U/L (<50)

Smooth muscle antibody Detected 1:200 -


Tags
Anti mitochondrial antibody Not detected -

Serum IgG 23 g/L (6-13)

Serum IgA 1 g/L (0.8-3)

What is the cause of his liver dysfunction?

(Please select 1 option)



Alcohol excess


Autoimmune hepatitis


Brucellosis


Viral hepatitis


Wilson's disease

Submit answer
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A 30-year-old man was referred to outpatients because of
abnormal liver function tests.
Answer Statistics
He had attended his general practitioner complaining of lethargy
and feeling generally unwell. He worked at his
family's dairy
farm. He drank alcohol regularly, consuming on average 30 units per
week. He took thyroxine for Test Analysis
hypothyroidism. He denied drug abuse;
he had never had a blood transfusion and had never travelled
abroad.

On examination he looked generally well, he was not overtly


jaundiced, there were no signs of chronic liver
disease.

His tests revealed

Haemoglobin 142 g/L (130-180)

White cells 9.0 ×109/L (4-11)

Platelets 300 ×109/L (150-400)

MCV 102 fL (80-96)

Albumin 40 g/L (37-49)

Bilirubin 35 μmol/L (1-22)

Alanine transferase 210 U/L (5-35)


Score:
Alkaline phosphatase 140 U/L (45-105)
Total Answered:
Gamma gluteryltransferase 120 U/L (<50)

Smooth muscle antibody Detected 1:200 -


Feedback
Anti mitochondrial antibody Not detected -

Serum IgG 23 g/L (6-13) Question Navigator


Serum IgA 1 g/L (0.8-3)

Revision Notes
What is the cause of his liver dysfunction?

(Please select 1 option) Tags


Alcohol excess
Incorrect answer selected

Autoimmune hepatitis
This is the correct answer


Brucellosis


Viral hepatitis


Wilson's disease

This man does drink but in the absence of other liver disease he
is unlikely to run into trouble with 30 units per
week. The raised
mean corpuscular volume (MCV) may point to alcohol excess but it is
not specific and his
thyroid status may be playing a role in
this.

There is nothing in the history to point to hepatitis C virus


(HCV) or hepatitis B virus (HBV) but both hepatitis A
and E are
faecal-oral spread and should be considered.

Brucellosis would go along with the contact with cattle but the
clinical features do not fit and it is very rare in the
United
Kingdom.
Wilson's should be looked for in this situation but the
antibody (+SMA) and immunoglobulin (high IgG) point to
autoimmune
hepatitis (AH).

Having one autoimmune disease (hypothyroidism) should also raise


the suspicion of another.

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Work Smart
A 50-year-old lady was seen in the clinic having been found by
her general practitioner to have deranged liver
Test Analysis
function.

She had been asymptomatic and felt very well. She had a past
history Graves' hyperthyroidism which had been
treated with
radio iodine and she was now managed with thyroxine.

On examination she had palmar erythema, and several spider


naevi. The rest of the examination was
unremarkable.

Haemoglobin 132 g/L (115-165)

White cells 9.0 ×109/L (4-11)

Platelets 110 ×109/L (150-400)

MCV 92 fL (80-96)

Albumin 36 g/L (37-49)

Bilirubin 28 µmol/L (1-22)


Score:
Alanine aminotransferase 50 U/L (5-35)

Alkaline phosphatase 400 U/L (45-105) Total Answered:

Gamma gluteryltransferase 490 U/L (<50)

Smooth muscle antibody Not detected


Question Navigator

Anti mitochondrial antibody Detected


Tags
What treatment option could you offer this lady?

(Please select 1 option)



Azathioprine


Cholestyramine


Interferon alpha


Prednisolone


Ursodeoxycholic acid

Submit answer
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A 50-year-old lady was seen in the clinic having been found by
her general practitioner to have deranged liver
Answer Statistics
function.

She had been asymptomatic and felt very well. She had a past
history Graves' hyperthyroidism which had been Test Analysis
treated with
radio iodine and she was now managed with thyroxine.

On examination she had palmar erythema, and several spider


naevi. The rest of the examination was
unremarkable.

Haemoglobin 132 g/L (115-165)

White cells 9.0 ×109/L (4-11)

Platelets 110 ×109/L (150-400)

MCV 92 fL (80-96)

Albumin 36 g/L (37-49)

Bilirubin 28 µmol/L (1-22)

Alanine aminotransferase 50 U/L (5-35)

Alkaline phosphatase 400 U/L (45-105)


Score:
Gamma gluteryltransferase 490 U/L (<50)
Total Answered:
Smooth muscle antibody Not detected

Anti mitochondrial antibody Detected


Feedback
What treatment option could you offer this lady?

(Please select 1 option) Question Navigator


Azathioprine
Incorrect answer selected
Revision Notes

Cholestyramine


Interferon alpha
Tags

Prednisolone

Ursodeoxycholic acid
This is the correct answer

This story suggests primary biliary cirrhosis (PBC) (middle aged


female history of autoimmunity with cholestatic
liver function
tests and a positive AMA).

Cholestyramine is used with itching of cholestasis but as this


lady is asymptomatic, there is, despite extensive
research, no role
for immune suppression in PBC.

The mainstay of therapy is urso which improves blood tests but


otherwise its benefits are modest.

There is no role for interferon alpha, prednisolone or


azathioprine.

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Work Smart MRCP Part 2 Written 2007 April

A 66-year-old lady presents to her GP with a two day history of


intermittent nose bleeds. She is otherwise
Test Analysis
relatively fit and well
with only hypertension as significant in her past medical
history.

On examination her blood pressure is 135/86, cardiovascular and


chest examination unremarkable. Her
abdomen is soft and non-tender,
with no masses or enlarged organs. She has numerous bruises, mainly
over
her limbs but also over her trunk and a petechial rash over
her shins.

The GP arranges some further investigations which are shown


below:

Haemoglobin 110 g/L (115-165)

MCV 83 fL (80-96)

White cell count 26.9 ×109/L (4-11)

Platelets 15 ×109/L (150-400)

Blood film: Thrombocytopenia with platelet anisocytosis. There


are numerous lymphocytes on the film, they are
mature with high
nuclear: cytoplasmic ratio and numerous smear cells present.
Score:
Manual differential:
Total Answered:
Neutrophils 4.3 ×109/L (1.5-7)

Lymphocytes 22.0 ×109/L (1.5-4) Question Navigator


Monocytes 0.4 ×109/L (0-0.8)
Tags
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1)

What would you advise as the next step in her management?

(Please select 1 option)



Chemotherapy and intravenous immunoglobulin


Intravenous immunoglobulin only


Observation


Platelet transfusion


Prednisolone treatment only

Submit answer
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Work Smart MRCP Part 2 Written 2007 April

A 66-year-old lady presents to her GP with a two day history of


intermittent nose bleeds. She is otherwise
Answer Statistics
relatively fit and well
with only hypertension as significant in her past medical
history.

On examination her blood pressure is 135/86, cardiovascular and


chest examination unremarkable. Her Test Analysis
abdomen is soft and non-tender,
with no masses or enlarged organs. She has numerous bruises, mainly
over
her limbs but also over her trunk and a petechial rash over
her shins.

The GP arranges some further investigations which are shown


below:

Haemoglobin 110 g/L (115-165)

MCV 83 fL (80-96)

White cell count 26.9 ×109/L (4-11)

Platelets 15 ×109/L (150-400)

Blood film: Thrombocytopenia with platelet anisocytosis. There


are numerous lymphocytes on the film, they are
mature with high
nuclear: cytoplasmic ratio and numerous smear cells present.

Manual differential:

Neutrophils 4.3 ×109/L (1.5-7) Score:

Lymphocytes 22.0 ×109/L (1.5-4) Total Answered:

Monocytes 0.4 ×109/L (0-0.8)


Feedback
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Question Navigator

What would you advise as the next step in her management?


Revision Notes
(Please select 1 option)
Chemotherapy and intravenous immunoglobulin
Correct Tags

Intravenous immunoglobulin only


Observation


Platelet transfusion


Prednisolone treatment only

The candidate needs to recognise that this is a case of immune


thrombocytopenia (ITP) complicating chronic
lymphocytic leukaemia
(CLL).

The CLL is diagnosed on the lymphocyte count and film findings,


the description of the lymphoid cells is classic
for CLL. The
haemoglobin is normal but the platelet count is very low. If the
CLL was causing bone marrow
compromise, that is, thrombocytopenia
to this degree, then the haemoglobin would be low. Autoimmune

complications are common with CLL and this is the most likely
cause.

Indications to treat in CLL include:


Lymphocyte doubling time of less than 12 months
Disabling B symptoms
Immune complications, for example, ITP, autoimmune
haemolysis
Bone marrow compromise (stage C disease).

Given this lady has an autoimmune complication secondary to her


CLL she requires treatment for the CLL.

The choice of chemotherapy agent will depend on any existing


co-morbidities; in this case as she is fit and well a
combination
of fludarabine and cyclophosphamide is indicated. However it will
take time for this to have an
effect and switch off the autoimmune
complication.

In the interim she has bleeding problems and a very low platelet
count, so you would also use some intravenous
immunoglobulin to
produce a rapid rise in the platelet, it usually has effect in
about 48 hours.

In ITP, platelets would only be indicated for life threatening


bleeding (or platelet count <10 ×109/L)

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Work Smart MRCP Part 2 Written 2007 April

A 49-year-old gentleman presents to the GP with tiredness and


headaches.
Test Analysis
He also reports an itchy rash after bathing. He has been an
ex-smoker for five years, but prior to that had
smoked for 20
years. He is under considerable stress in his office job and has
been taking a homeopathic
medicine for stress. He is on no other
medication. There is a family history of hypertension.

On examination he is plethoric. Cardiovascular examination and


auscultation of the chest is unremarkable. On
palpation of the
abdomen the GP could palpate the spleen two finger breadths below
the left costal margin. His
blood pressure is 138/89 mmHg.

The GP refers him for a number of investigations. The results


are as follows:

Full blood count:

Haemoglobin 208 g/L (130-180)

Haematocrit 0.61 (0.40-0.52)

White cell count 18.0 ×109/L (4-11)

15.5 ×109/L
Score:
Neutrophils (1.5-7)
Total Answered:
Lymphocytes 2.0 ×109/L (1.5-4)

Monocytes 0.2 ×109/L (0-0.8)


Question Navigator
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1)


Tags

Platelet count 550 ×109/L (150-400)

Haematinics:

Serum ferritin 15 g/L (15-300)

Serum B12 900 ng/L (160-760)

Serum folate 5.0 g/L (2.0-11.0)

Coagulation screen:

Prothrombin time 13.0 s (11.5-15.5)

Activated partial thromboplastin time 35 s (30-40)

Fibrinogen 4.0 g/L (1.8-5.4)

Chemistry:

Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9)

Serum urea 7.5 mmol/L (2.5-7.5)

Serum creatinine 98 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)


Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.0 mmol/L (0.8-1.4)

Serum corrected calcium 2.3 mmol/L (2.2-2.6)

What is the diagnosis?

(Please select 1 option)



Apparent erythrocytosis due to the homeopathic medicine


Chronic myeloid leukaemia


Idiopathic erythrocytosis


Polycythaemia vera


Secondary erythrocytosis

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Work Smart MRCP Part 2 Written 2007 April

A 49-year-old gentleman presents to the GP with tiredness and


headaches.
Answer Statistics
He also reports an itchy rash after bathing. He has been an
ex-smoker for five years, but prior to that had
smoked for 20
years. He is under considerable stress in his office job and has
been taking a homeopathic Test Analysis
medicine for stress. He is on no other
medication. There is a family history of hypertension.

On examination he is plethoric. Cardiovascular examination and


auscultation of the chest is unremarkable. On
palpation of the
abdomen the GP could palpate the spleen two finger breadths below
the left costal margin. His
blood pressure is 138/89 mmHg.

The GP refers him for a number of investigations. The results


are as follows:

Full blood count:

Haemoglobin 208 g/L (130-180)

Haematocrit 0.61 (0.40-0.52)

White cell count 18.0 ×109/L (4-11)

Neutrophils 15.5 ×109/L (1.5-7)

Lymphocytes 2.0 ×109/L (1.5-4)


Score:
Monocytes 0.2 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.2 ×109/L (0-0.1)


Feedback

Platelet count 550 ×109/L (150-400)


Question Navigator
Haematinics:
Revision Notes
Serum ferritin 15 g/L (15-300)

Serum B12 900 ng/L (160-760)


Tags
Serum folate 5.0 g/L (2.0-11.0)

Coagulation screen:

Prothrombin time 13.0 s (11.5-15.5)

Activated partial thromboplastin time 35 s (30-40)

Fibrinogen 4.0 g/L (1.8-5.4)

Chemistry:

Serum sodium 137 mmol/L (137-144)

Serum potassium 4.8 mmol/L (3.5-4.9)

Serum urea 7.5 mmol/L (2.5-7.5)

Serum creatinine 98 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 90 U/L (45-105)


Serum total bilirubin 22 µmol/L (1-22)

Serum phosphate 1.0 mmol/L (0.8-1.4)

Serum corrected calcium 2.3 mmol/L (2.2-2.6)

What is the diagnosis?

(Please select 1 option)


Apparent erythrocytosis due to the homeopathic medicine
Incorrect answer selected


Chronic myeloid leukaemia


Idiopathic erythrocytosis

Polycythaemia vera
This is the correct answer


Secondary erythrocytosis

There is a high haemoglobin (Hb) and haematocrit (HCT) -


therefore there is polycythaemia. This is the most
significant
abnormality. There is also a mild neutrophilia and thrombocytosis
with a slightly raised basophil
count.

Absolute erythrocytosis, as opposed to apparent, is defined as


an HCT greater than 0.60 in males and HCT
greater than 0.56 in
females. We have therefore ruled out apparent erythrocytosis in
this man.

We are therefore left trying to decide if this is


Primary erythrocytosis (polycythaemia vera [PV])
Secondary erythrocytosis (due to some congenital or acquired
cause) or
Idiopathic erythrocytosis (those with an absolute polycythaemia
but not primary and no secondary
cause).

The diagnosis favours polcythaemia vera as opposed to secondary


or idiopathic for the following reasons:
Low serum ferritin is more common in PV than secondary
High B12 is characteristic of PV due to transcobalamin release
from an increased granulocytic mass
In the absence of liver disease a palpable spleen is a reliable
sign of PV
The pruritus is typically aquagenic as described here.

In PV, the white cell count and platelet count can be


elevated.

In chronic myeloid leukaemia you may expect a higher white cell


count and would not see the polycythaemia.

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Work Smart MRCP Part 2 Written 2007 April

A 38-year-old gentleman is seen on the haematology day unit 55


days post sibling allogeneic bone marrow
Test Analysis
transplant for acute
myeloid leukaemia.

He has been doing relatively well until three days ago when he
first noticed that his urine was dark coloured.
Then he developed a
fever 24 hours ago and subsequently has become very confused.

On examination he is unwell with a temperature of 39°C, pulse


110 beats per minute and regular, normotensive.
His sclerae are
yellow, cardiovascular, chest and abdomen are unremarkable.
Neurological examination shows
him to be confused and very drowsy
but no focal abnormality detected. His blood results are shown
below.

Full blood count:

Haemoglobin 62 g/L (130-180)

White cell count 12.0 ×109/L (4-11)

Neutrophils 9.6 ×109/L (1.5-7)

Lymphocytes 2.0 ×109/L (1.5-4)


Score:
Monocytes 0.2 ×109/L (0-0.8)
Total Answered:
Eosinophils 0.1 ×109/L (0.04-0.4)

Basophils 0.1 ×109/L (0-0.1) Question Navigator


Platelet count 26 ×109/L (150-400)
Tags
Reticulocyte count 16.0%

Blood film: There is gross red cell fragmentation with


polychromasia. There are numerous spherocytes and
nucleated red
cells. The white cells are reactive.

Coagulation screen:

Prothrombin time 15.0 s (11.5-15.5)

Activated partial thromboplastin time 35 s (30-40)

Fibrinogen 6.6 g/L (1.8-5.4)

D-dimer screen <0.5 mg/L (<0.5)

Serum sodium 137 mmol/L (137-144)

Serum potassium 5.6 mmol/L (3.5-4.9)

Serum urea 18.0 mmol/L (2.5-7.5)

Serum creatinine 231 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 120 U/L (45-105)

Serum total bilirubin 100 µmol/L (1-22)

Serum lactate dehydrogenase 1618 U/L (10-250)

Serum magnesium 1.0 mmol/L (0.75-1.05)

Serum phosphate 1.38 mmol/L (0.8-1.4)

Serum corrected calcium 2.5 mmol/L (2.2-2.6)


Blood ciclosporin level: 420 nmol/L

What is the most likely diagnosis?

(Please select 1 option)



Autoimmune haemolysis


Autoimmune thrombocytopenia


Disseminated intravascular coagulation (DIC)


Relapse of his leukaemia


Thrombotic thrombocytopenic purpura (TTP)

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Work Smart MRCP Part 2 Written 2007 April

A 38-year-old gentleman is seen on the haematology day unit 55


days post sibling allogeneic bone marrow
Answer Statistics
transplant for acute
myeloid leukaemia.

He has been doing relatively well until three days ago when he
first noticed that his urine was dark coloured. Test Analysis
Then he developed a
fever 24 hours ago and subsequently has become very confused.

On examination he is unwell with a temperature of 39°C, pulse


110 beats per minute and regular, normotensive.
His sclerae are
yellow, cardiovascular, chest and abdomen are unremarkable.
Neurological examination shows
him to be confused and very drowsy
but no focal abnormality detected. His blood results are shown
below.

Full blood count:

Haemoglobin 62 g/L (130-180)

White cell count 12.0 ×109/L (4-11)

Neutrophils 9.6 ×109/L (1.5-7)

Lymphocytes 2.0 ×109/L (1.5-4)

Monocytes 0.2 ×109/L (0-0.8)

Eosinophils 0.1 ×109/L (0.04-0.4) Score:

Basophils 0.1 ×109/L (0-0.1) Total Answered:

Platelet count 26 ×109/L (150-400)


Feedback
Reticulocyte count 16.0%

Blood film: There is gross red cell fragmentation with


polychromasia. There are numerous spherocytes and Question Navigator
nucleated red
cells. The white cells are reactive.

Coagulation screen: Revision Notes

Prothrombin time 15.0 s (11.5-15.5)


Tags
Activated partial thromboplastin time 35 s (30-40)

Fibrinogen 6.6 g/L (1.8-5.4)

D-dimer screen <0.5 mg/L (<0.5)

Serum sodium 137 mmol/L (137-144)

Serum potassium 5.6 mmol/L (3.5-4.9)

Serum urea 18.0 mmol/L (2.5-7.5)

Serum creatinine 231 µmol/L (60-110)

Serum aspartate aminotransferase 30 U/L (1-31)

Serum alkaline phosphatase 120 U/L (45-105)

Serum total bilirubin 100 µmol/L (1-22)

Serum lactate dehydrogenase 1618 U/L (10-250)

Serum magnesium 1.0 mmol/L (0.75-1.05)

Serum phosphate 1.38 mmol/L (0.8-1.4)

Serum corrected calcium 2.5 mmol/L (2.2-2.6)


Blood ciclosporin level: 420 nmol/L

What is the most likely diagnosis?

(Please select 1 option)


Autoimmune haemolysis
Incorrect answer selected


Autoimmune thrombocytopenia


Disseminated intravascular coagulation (DIC)


Relapse of his leukaemia

Thrombotic thrombocytopenic purpura (TTP)


This is the correct answer

There is evidence of microangiopathic haemolytic anaemia (MAHA)


- low Hb, high reticulocytes and
polychromasia, elevated lactate
dehydrogenase (LDH) - there is also thrombocytopenia. This really
rules out
autoimmune haemolysis, autoimmune thrombocytopenia or a
relapse of his leukaemia.

This leaves either disseminated intravascular coagulation (DIC)


or thrombotic thrombocytopenic purpura (TTP).

In TTP there is classically a pentad:


1. Microangiopathic haemolytic anaemia (MAHA)
2. Low platelets
3. Abnormal neurology
4. Renal impairment
5. Fever

Very often the pentad is not present, but this patient really
has all five of the above.

The predominant histological abnormality in TTP is the formation


of platelet microvascular thrombi, with the renal
and cerebral
circulations primarily affected.

The excessive platelet aggregation is mediated by ultra-large


von Willebrand factor multimers (ULVWF). These
are not normally
present in the circulation since they are degraded to smaller
subunits by a protease known as
ADAMTS 13. This is known to be
deficient in TTP.

There are a number of causes of acquired TTP including post bone


marrow transplant and ciclosporin, which are
likely to be the cause
in this patient.

The treatment of TTP is single volume daily plasma exchange with


pathogen reduced plasma,
methylprednisolone 1 gram daily for three
days.

Anti-platelet agents can be considered when platelets are


greater than 100. Ciclosporin should be stopped and
alternative
graft versus host disease prophylaxis used.

Against DIC in this instance is the normal coagulation screen,


because by definition DIC is a consumptive
coagulopathy.

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Work Smart MRCP Part 2 Written 2007 April

A 32-year-old female suffers multiple traumas in a road traffic


accident including fracture of her pelvis, right femur
Test Analysis
and left
humerus. She is taken immediately to theatre for surgery.

During surgery and immediately post operatively she has


extensive life threatening bleeding and requires 24
units of red
cells, 6 units of platelets and 16 units of fresh frozen plasma.
She undergoes a stormy post
operative recovery initially on the
intensive care unit and then the general ward.

You are called to see her five days post operatively. She is
receiving a blood transfusion and has spiked a
temperature 45
minutes after starting a unit of red cells. Her temperature is
38.2°C and blood pressure is 110/70
mmHg and heart rate is 98 bpm.
The red cell transfusion is stopped and patient identity checked
and the unit of
blood is checked against her identity, all appear
to be correct.

A transfusion reaction investigation is initiated by the


hospital's transfusion practitioner and transfusion team. The

patient is monitored closely and remains well over the next 24


hours. Her temperature returned to normal within
20 minutes of
stopping the transfusion.

Further information emerges the next day following


investigations. The patient is blood group A rhesus D
negative, and
the unit of blood being transfused during the above reported
reaction was of the correct group and
Score:
cross match compatible.
However, it appears that during her emergency surgery and
subsequent bleeding she
received 2 units of rhesus D positive
platelets. Total Answered:

What action do you advise now?


Question Navigator
(Please select 1 option)

Administer anti D immunoglobulin
Tags

Advise patient that this is only likely to be of consequence should she become pregnant in the future.


Advise that this is of no further consequence to her


Arrange red cell exchange transfusion


Measure anti D titre in patient

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Work Smart MRCP Part 2 Written 2007 April

A 32-year-old female suffers multiple traumas in a road traffic


accident including fracture of her pelvis, right femur
Answer Statistics
and left
humerus. She is taken immediately to theatre for surgery.

During surgery and immediately post operatively she has


extensive life threatening bleeding and requires 24 Test Analysis
units of red
cells, 6 units of platelets and 16 units of fresh frozen plasma.
She undergoes a stormy post
operative recovery initially on the
intensive care unit and then the general ward.

You are called to see her five days post operatively. She is
receiving a blood transfusion and has spiked a
temperature 45
minutes after starting a unit of red cells. Her temperature is
38.2°C and blood pressure is 110/70
mmHg and heart rate is 98 bpm.
The red cell transfusion is stopped and patient identity checked
and the unit of
blood is checked against her identity, all appear
to be correct.

A transfusion reaction investigation is initiated by the


hospital's transfusion practitioner and transfusion team. The

patient is monitored closely and remains well over the next 24


hours. Her temperature returned to normal within
20 minutes of
stopping the transfusion.

Further information emerges the next day following


investigations. The patient is blood group A rhesus D
negative, and
the unit of blood being transfused during the above reported
reaction was of the correct group and
cross match compatible.
However, it appears that during her emergency surgery and
subsequent bleeding she
received 2 units of rhesus D positive
platelets.
Score:
What action do you advise now?
Total Answered:
(Please select 1 option)
Administer anti D immunoglobulin
Incorrect answer selected
Feedback
Advise patient that this is only likely to be of consequence should she become pregnant in the future.
This is the
correct answer
Question Navigator

Advise that this is of no further consequence to her


Arrange red cell exchange transfusion Revision Notes

Measure anti D titre in patient
Tags

The transfusion reaction described is likely to be a febrile


transfusion reaction. Following the investigations there
does not
appear to be an ABO incompatible transfusion. Her temperature
settles after stopping the transfusion
and she is not that
unwell.

Febrile transfusion reactions are common and are due to anti HLA
antibodies in recipient serum or granulocyte
specific antibodies
(for example, sensitisation during previous pregnancy or previous
blood transfusion).

All hospitals have (or should have) a transfusion team and


committee to report, amongst other things, transfusion
related
events and reactions to SHOT (serious hazards of transfusion) and
SABRE (serious adverse blood
related events).

This patient has been given Rh D positive platelets when she is


RhD negative, she is therefore by now (five days
later) likely to
have made immune anti-D. It is very often necessary to give D
positive platelets to D negative
people due to platelet
shortage.

If the recipient of this mismatch is a female of child bearing


age then prophylactic anti- D should be administered
with the
platelets to prevent production of immune anti- D. In this case it
is too late to administer the anti-D.
Should she become pregnant in
the future, the immune anti-D she has made can cross the placenta
and cause
haemolytic disease of the fetus/newborn, if the baby is D
positive, and this can be life threatening to the baby.
Therefore any pregnancies would have to be managed by an
obstetrician with special interest in fetal medicine
and the baby
closely monitored and the levels of anti-D in the mother
monitored.

Further Reading:

British Society for Haematology. Haematology Guidelines: Transfusion.

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You are called to the Emergency department to assess a
21-year-old student who has presented with bloody
Test Analysis
diarrhoea.

The diarrhoea started two weeks previously and was associated


with increasing nausea and malaise and mild
swelling of the lower
limbs. She was having difficulty passing urine. She had eaten steak
from the local butcher
at a friend's barbecue the day before
developing diarrhoea.

On examination she was pale with evidence of petechiae over her


legs. Her face appeared puffy. Blood pressure
was 160/95 mmHg. She
was apyrexial but had a tachycardia and crackles on inspiration at
both lung bases.
There was an old appendicectomy scar in the right
iliac fossa.

Investigations showed:

Haemoglobin 85 g/L (115-165)

White cell count 13.2 ×109/L (4-11)

Neutrophils 9.5 ×109/L (1.5-7)

Platelets 35 ×109/L (150-400) Score:

PT 12 s (11.5-15.5) Total Answered:

APTT 34 s (30-40)

Fibrinogen 4 g/L (1.8-5.4) Question Navigator


Serum sodium 139 mmol/L (137-144)
Tags
Serum potassium 6.1 mmol/L (3.5-4.9)

Serum urea 40 mmol/L (2.5-7.5)

Serum creatinine 411 µmol/L (60-110)

Serum albumin 27 g/L (37-49)

Dipstick urine Blood ++ Protein +

What is the single most important next investigation to


determine the diagnosis?

(Please select 1 option)



ASO titres


Blood film analysis


Renal tract ultrasound


Transthoracic echocardiogram


Urine microscopy

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You are called to the Emergency department to assess a
21-year-old student who has presented with bloody
Answer Statistics
diarrhoea.

The diarrhoea started two weeks previously and was associated


with increasing nausea and malaise and mild Test Analysis
swelling of the lower
limbs. She was having difficulty passing urine. She had eaten steak
from the local butcher
at a friend's barbecue the day before
developing diarrhoea.

On examination she was pale with evidence of petechiae over her


legs. Her face appeared puffy. Blood pressure
was 160/95 mmHg. She
was apyrexial but had a tachycardia and crackles on inspiration at
both lung bases.
There was an old appendicectomy scar in the right
iliac fossa.

Investigations showed:

Haemoglobin 85 g/L (115-165)

White cell count 13.2 ×109/L (4-11)

Neutrophils 9.5 ×109/L (1.5-7)

Platelets 35 ×109/L (150-400)

PT 12 s (11.5-15.5)
Score:
APTT 34 s (30-40)
Total Answered:
Fibrinogen 4 g/L (1.8-5.4)

Serum sodium 139 mmol/L (137-144)


Feedback
Serum potassium 6.1 mmol/L (3.5-4.9)

Serum urea 40 mmol/L (2.5-7.5)


Question Navigator
Serum creatinine 411 µmol/L (60-110)

Serum albumin 27 g/L (37-49) Revision Notes


Dipstick urine Blood ++ Protein +

Tags
What is the single most important next investigation to
determine the diagnosis?

(Please select 1 option)


ASO titres
Incorrect answer selected

Blood film analysis


This is the correct answer


Renal tract ultrasound


Transthoracic echocardiogram


Urine microscopy

This patient has haemolytic uraemic syndrome (HUS).

It typically presents with a triad of:


Acute renal failure (ARF)
Microangiopathic haemolytic anaemia, and
Thrombocytopenia with normal clotting.

HUS is a complication of infection with verocytotoxin producing


Escherichia coli usually of the serotype 0157:H7.
Toxins produced in the intestine enter the blood and bind to
endothelial cells in target organs. Endothelial cell
damage leads
to platelet and fibrin deposition with resultant fragmentation of
circulating red blood cells and
microvascular occlusion.

The syndrome has also been reported after infections with


coxsackie, echovirus and Shigella.

HUS is characterised by the sudden onset of haemolytic anaemia


with fragmentation of red blood cells,
thrombocytopenia and acute
renal failure after a prodromal illness of acute gastroenteritis
often with bloody
diarrhoea.

Clinical signs include increasing pallor, haematuria, oliguria


and purpura. Jaundice is occasionally seen.
Hypertension may be
present.

Typical results show an anaemia, thrombocytopenia, and often a


neutrophilia. Blood film shows fragmented
erythrocytes.

Urea and electrolytes are typical of acute renal failure. There


is normal coagulation and fibrinogen.

Neurological complications include:


Stroke, seizure and coma occur in 25% of patients
Rarely pancreatitis, and
Pleural and pericardial effusions.

Approximately 5% of patients will develop end stage renal


failure.

Long term renal sequelae range from proteinuria to chronic renal


failure.

Therapy is supportive with:


Correction of anaemia
Correction of uraemia by early dialysis
Strict fluid balance
Treatment of hypertension.

Major differential diagnosis is:


1. Sepsis with DIC - presents with abnormalities of clotting
parameters.
2. TTP - thrombotic thrombocytopenic purpura presents with
microangiopathic haemolytic anaemia,
thrombocytopenic purpura,
neurologic abnormalities, fever, and renal disease.

Renal abnormalities tend to be more severe in HUS.

Although once considered variants of a single syndrome, recent


evidence suggests that the pathogenesis of TTP
and HUS is
different. Patients with TTP lack a plasma protease that is
responsible for the breakdown of von
Willebrand factor (vWF)
multimers and these accumulate in the plasma. The activity of this
protease is normal in
patients with HUS.

Until the test for vWF protease activity becomes available,


differentiation between HUS and TTP is based on the
presence of
central nervous system involvement in TTP and the more severe renal
involvement in HUS.

In HUS 90% of patients are children and a history of prodromal


diarrhoeal illness is more common.

The therapy of choice for TTP is plasma exchange with fresh


frozen plasma.

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A 68-year-old woman was admitted to hospital with severe acute
dyspnoea. She denied having any chest pain
Test Analysis
but said that she had
become progressively breathless over the past three months.

On examination her pulse was 120 beats per minute and regular.
Her blood pressure was 95/55 mmHg and her
jugular venous pressure
was elevated to the angle of the jaw. Her heart sounds were normal.
Auscultation of her
chest revealed bilateral fine inspiratory
crackles to the mid zones. She had haemorrhages in both fundi.

Investigations revealed:

Haemoglobin 56 g/L (115-165)

Haematocrit 0.19 (0.36-0.47)

MCV 118 fL (80-96)

MCH 33.0 pg (28-32)

White cell count 3.4 ×109/L (4-11)

Platelets 95 ×109/L (150-400)


Score:
Serum vitamin B12 Result pending

Serum folate Result pending Total Answered:

The ECG showed left bundle branch block, which had been
documented previously.
Question Navigator
She is given 80 mg of intravenous furosemide which results in an
excellent diuresis.

What is the next most appropriate immediate step in her


management? Tags
(Please select 1 option)

Blood transfusion


Bone marrow aspiration


Start intramuscular vitamin B12 and oral folic acid


Start oral ferrous sulphate


Thrombolyse with t-PA

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A 68-year-old woman was admitted to hospital with severe acute
dyspnoea. She denied having any chest pain
Answer Statistics
but said that she had
become progressively breathless over the past three months.

On examination her pulse was 120 beats per minute and regular.
Her blood pressure was 95/55 mmHg and her Test Analysis
jugular venous pressure
was elevated to the angle of the jaw. Her heart sounds were normal.
Auscultation of her
chest revealed bilateral fine inspiratory
crackles to the mid zones. She had haemorrhages in both fundi.

Investigations revealed:

Haemoglobin 56 g/L (115-165)

Haematocrit 0.19 (0.36-0.47)

MCV 118 fL (80-96)

MCH 33.0 pg (28-32)

White cell count 3.4 ×109/L (4-11)

Platelets 95 ×109/L (150-400)

Serum vitamin B12 Result pending

Serum folate Result pending


Score:
The ECG showed left bundle branch block, which had been
documented previously.
Total Answered:
She is given 80 mg of intravenous furosemide which results in an
excellent diuresis.

What is the next most appropriate immediate step in her


management? Feedback
(Please select 1 option)
Question Navigator
Blood transfusion
Correct


Bone marrow aspiration
Revision Notes

Start intramuscular vitamin B12 and oral folic acid


Start oral ferrous sulphate Tags


Thrombolyse with t-PA

The clinical picture represents severe megaloblastic anaemia


with cardiac failure.

The question asks about immediate management. Although the


anaemia has been developing slowly, she has
become acutely
haemodynamically compromised. In such circumstances it would be
most appropriate to
transfuse the patient. This would need to be
done very cautiously with diuretic cover.

She will clearly need to start an intensive course of


intramuscular vitamin B12 and oral folic acid as well, but this
is
less important in the hyperacute situation where there is a risk of
the patient dying from anaemia.

Giving oral folic acid without vitamin B12 would be hazardous


and could precipitate subacute combined
degeneration of the spinal
cord.

Transfusion may also be hazardous in a patient with severe


congestive cardiac failure (CCF).

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A 36-year-old woman presented to hospital with a three day
history of pain and swelling in her right leg.
Test Analysis
Ten years previously she was diagnosed as having a deep vein
thrombosis of the right calf after returning from
her honeymoon in
Australia. Three years previously she was treated by her general
practitioner for pleurisy.

She has a history of recurrent 'water' infections: for


two years she has developed intermittent bouts of flank pain
and
has also noted that she occasionally passes dark urine in the
morning. This has recently been investigated
by her general
practitioner. An ultrasound scan of her renal tract and an
intravenous pyelogram were normal.

She works as a cashier in a building society and lives with her


husband and two children. There is no recent
history of travel. She
smokes 10 cigarettes daily and occasionally drinks alcohol. She
does not take any regular
medication. On examination she appears
well. Temperature 37.9°C. The right calf is swollen, red and
tender.

Investigations show:

Haemoglobin 85 g/L (115-165)

White cell count 2.1 ×109/L (4-11)

Neutrophils 35% - Score:


Lymphocytes 60% - Total Answered:
Monocytes 4% -

Platelets 79 ×109/L (150-400) Question Navigator


Sodium 141 mmol/L (137-144)
Tags
Potassium 4.2 mmol/L (3.5-4.9)

Urea 5.0 mmol/L (2.5-7.5)

Creatinine 70 µmol/L (60-110)

Bilirubin 35 µmol/L (1-22)

Alk phos 170 U/L (45-105)

AST 25 U/L (1-31)

Coagulation screen normal.

What is the most likely diagnosis?

(Please select 1 option)



Antiphospholipid syndrome


Buerger's disease


Paroxysmal nocturnal haemoglobinuria


Polyarteritis nodosa


Rhabdomyolysis

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Work Smart
A 36-year-old woman presented to hospital with a three day
history of pain and swelling in her right leg.
Answer Statistics
Ten years previously she was diagnosed as having a deep vein
thrombosis of the right calf after returning from
her honeymoon in
Australia. Three years previously she was treated by her general
practitioner for pleurisy. Test Analysis
She has a history of recurrent 'water' infections: for
two years she has developed intermittent bouts of flank pain
and
has also noted that she occasionally passes dark urine in the
morning. This has recently been investigated
by her general
practitioner. An ultrasound scan of her renal tract and an
intravenous pyelogram were normal.

She works as a cashier in a building society and lives with her


husband and two children. There is no recent
history of travel. She
smokes 10 cigarettes daily and occasionally drinks alcohol. She
does not take any regular
medication. On examination she appears
well. Temperature 37.9°C. The right calf is swollen, red and
tender.

Investigations show:

Haemoglobin 85 g/L (115-165)

White cell count 2.1 ×109/L (4-11)

Neutrophils 35% -

Lymphocytes 60% -

Monocytes 4% -
Score:

Platelets 79 ×109/L (150-400) Total Answered:

Sodium 141 mmol/L (137-144)


Feedback
Potassium 4.2 mmol/L (3.5-4.9)

Urea 5.0 mmol/L (2.5-7.5)


Question Navigator
Creatinine 70 µmol/L (60-110)

Bilirubin 35 µmol/L (1-22) Revision Notes


Alk phos 170 U/L (45-105)

AST 25 U/L (1-31) Tags

Coagulation screen normal.

What is the most likely diagnosis?

(Please select 1 option)


Antiphospholipid syndrome
Incorrect answer selected


Buerger's disease

Paroxysmal nocturnal haemoglobinuria


This is the correct answer


Polyarteritis nodosa


Rhabdomyolysis

Paroxysmal nocturnal haemoglobinuria (PNH) is an acquired clonal


defect of red cells that results in production
of erythrocytes with
an increased susceptibility to lysis by complement. The disorder
usually presents in young
adults and probably arises from a
mutation in a single stem cell and is frequently associated with
other stem cell
disorders (notably aplastic anaemia).
Intermittent passage of bloody urine is the classical feature of
the disease; why this should occur more frequently
at night is
uncertain.

Patients are more likely to be diagnosed following:


Investigation of anaemia (due to chronic blood loss)
Pancytopenia (mild neutropenia and thrombocytopenia are
frequently present), or
Recurrent thrombotic episodes.

The prothrombotic tendency is probably caused by


complement-induced platelet aggregation.

The diagnosis can be established by flow cytometry,


demonstrating lack of the erythrocyte membrane proteins
CD59 and
decay accelerating factor (DAF), or by employing Ham's acid
lysis test. In Ham's test, acidified serum
(pH 6.2) is added to
blood: PNH cells, but not normal cells, will be lysed.

Further Reading:

Medscape Reference. Paroxysmal Nocturnal Hemoglobinuria.

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A previously fit 30-year-old male presents with a two month
history of weight loss, tiredness and nausea.
Test Analysis
Investigations show:
Haemoglobin 105 g/L (130-180)

MCV 88 fL (80-96)

White cell count 6.0 ×109/L (4-11)

Platelets 450 ×109/L (150-400)

Serum sodium 130 mmol/L (137-144)

Serum potassium 5.7 mmol/L (3.5-4.9)

Serum urea 3.0 mmol/L (2.5-7.5)

Serum creatinine 78 µmol/L (60-110)

Serum total T4 55 nmol/L (50-150)

Serum TSH 8 mU/L (0.4-5)


Score:
Which of the following is the most useful diagnostic
investigation?
Total Answered:
(Please select 1 option)

Anti-thyroid peroxidase antibody titre Question Navigator

Free thyroxine concentration


Insulin tolerance test
Tags


Short Synacthen test


TRH test

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A previously fit 30-year-old male presents with a two month
history of weight loss, tiredness and nausea.
Answer Statistics
Investigations show:
Haemoglobin 105 g/L (130-180) Test Analysis
MCV 88 fL (80-96)

White cell count 6.0 ×109/L (4-11)

Platelets 450 ×109/L (150-400)

Serum sodium 130 mmol/L (137-144)

Serum potassium 5.7 mmol/L (3.5-4.9)

Serum urea 3.0 mmol/L (2.5-7.5)

Serum creatinine 78 µmol/L (60-110)

Serum total T4 55 nmol/L (50-150)

Serum TSH 8 mU/L (0.4-5)

Which of the following is the most useful diagnostic


investigation?

(Please select 1 option) Score:

Anti-thyroid peroxidase antibody titre


Incorrect answer selected Total Answered:


Free thyroxine concentration


Insulin tolerance test
Feedback

Short Synacthen test


This is the correct answer
Question Navigator

TRH test

Revision Notes
This patient presents with weight loss, tiredness and nausea. He
has hyponatraemia, hyperkalaemia and what
appears to be a mild
primary hypothyroidism. Tags

The diagnosis is likely to be Addison's (primary


hypoadrenalism) disease and the most appropriate test would be
a
short Synacthen test.

The link between Addison's and primary hypothyroidism is


that they are both conditions in the complex of
autoimmune
polyendocrine syndrome. Other possible associations of this cluster
would be
Type 1 diabetes
Vitiligo
Pernicious anaemia and
Chronic active hepatitis.

An insulin tolerance test is contraindicated in patients in whom


cortisol is less than 100 nmol/L.

A thyrotropin-releasing hormone (TRH) test is rarely performed


these days and really is an irrelevance.

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Work Smart MRCP Part 2 Written July 2011

A 26-year-old woman presented at 35 weeks of pregnancy with


profuse vaginal bleeding. She had suffered two
Test Analysis
previous
miscarriages.

She had a pulse of 95 beats per minute, blood pressure of 110/84


mmHg and no fetal heart sounds were
audible.

Investigations revealed:

Haemoglobin 98 g/L (115 - 165)

Platelets 66 ×109/L (150 - 400)

Prothrombin time 21 sec (11.5-15.5)

APTT 52 sec (30-40)

Fibrinogen 0.5 g/L (2-4)

What is the most appropriate next step in management?

(Please select 1 option) Score:



Antithrombin III infusion
Total Answered:

Fibrinogen replacement infusion (cryoprecipitate)


Intravenous heparin Question Navigator

Platelet transfusion
Tags

Transfusion of two units group O rhesus D negative blood

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Work Smart MRCP Part 2 Written July 2011

A 26-year-old woman presented at 35 weeks of pregnancy with


profuse vaginal bleeding. She had suffered two
Answer Statistics
previous
miscarriages.

She had a pulse of 95 beats per minute, blood pressure of 110/84


mmHg and no fetal heart sounds were Test Analysis
audible.

Investigations revealed:

Haemoglobin 98 g/L (115 - 165)

Platelets 66 ×109/L (150 - 400)

Prothrombin time 21 sec (11.5-15.5)

APTT 52 sec (30-40)

Fibrinogen 0.5 g/L (2-4)

What is the most appropriate next step in management?

(Please select 1 option)


Antithrombin III infusion
Incorrect answer selected

Fibrinogen replacement infusion (cryoprecipitate)


This is the correct answer Score:


Intravenous heparin Total Answered:


Platelet transfusion
Feedback

Transfusion of two units group O rhesus D negative blood

Question Navigator
The clinical picture is disseminated intravascular
coagulation.

When bleeding is the major problem, the aim is to maintain the


prothrombin and activated thromboplastin time at Revision Notes
a ratio of 1.5
times of the control and the fibrinogen level above 1 g/L.
Tags
Platelet transfusion is recommended if the count is less than 50
×109/L.

Anaemia is not very severe so in this case fibrinogen


replacement would be the appropriate first choice with
blood
transfusion an addition if bleeding continues and patient develops
hypovolaemic shock.

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The anticoagulation nurse phones you for advice.
Test Analysis
A 73-year-old lady is on warfarin for atrial fibrillation, her
normal INR range falls between 2.0-3.0. She has
recently been
started on clarithromycin for a chest infection; her INR has now
come back at greater than 9.0.

What should you advise?

(Please select 1 option)



Stop warfarin


Stop warfarin and administer vitamin K and fresh frozen plasma
(FFP)


Stop warfarin and convert to IV heparin (high risk of stroke)


Stop warfarin, administer vitamin K, re-start warfarin when INR
less than 5


Stop warfarin, stop clarithromycin and seek haematology advice

Score:
Submit answer
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Total Answered:

Question Navigator

Tags

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The anticoagulation nurse phones you for advice.
Answer Statistics
A 73-year-old lady is on warfarin for atrial fibrillation, her
normal INR range falls between 2.0-3.0. She has
recently been
started on clarithromycin for a chest infection; her INR has now
come back at greater than 9.0. Test Analysis
What should you advise?

(Please select 1 option)


Stop warfarin
Incorrect answer selected


Stop warfarin and administer vitamin K and fresh frozen plasma
(FFP)


Stop warfarin and convert to IV heparin (high risk of stroke)

Stop warfarin, administer vitamin K, re-start warfarin when INR


less than 5
This is the correct answer


Stop warfarin, stop clarithromycin and seek haematology advice

As per British Journal of Haematology (BJH) guidelines:

INR >8.0, no bleeding or minor bleeding:


Score:
Stop warfarin and give phytomenadione (vitamin K1)
2.5-5 mg by mouth using the intravenous preparation orally

(unlicensed use), or 0.5-1 mg by slow intravenous injection (if


complete reversal required 5-10 mg by slow Total Answered:
intravenous injection);
repeat dose of phytomenadione if INR still too high after 24 hours;
restart warfarin when
INR <5.0. Feedback
INR 5.0-8.0, no bleeding:

Stop warfarin.
Question Navigator

INR 5.0-8.0, minor bleeding:


Revision Notes
Stop warfarin and give phytomenadione (vitamin K1)
1-2.5 mg by mouth using the intravenous preparation orally

(unlicensed use); restart warfarin when INR <5.0. Tags


Unexpected bleeding at therapeutic levels:

Always investigate possibility of underlying cause, for example,


unsuspected renal or gastrointestinal tract
pathology.

Reference:

GP Notebook. Warfarin (prolonged INR).

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A 76-year-old retired steel industry employee is diagnosed with
renal cell carcinoma.
Test Analysis
It is noted that his full blood count is grossly abnormal:

Haemoglobin (Hb) 203 g/L (130-180)

Haematocrit (PCV) 0.60% (0.40-0.52)

Mean cell volume (MCV) 90 fL (84-96)

White cell count (WBC) 10 ×109/L (4.0-11.0)

Platelets 400 ×109/L (150-450)

Which of the following symptoms can be explained by the above


blood test results?

(Please select 1 option)



Onycholysis


Pruritus
Score:

Pyrexia
Total Answered:

Torticollis


Vitiligo
Question Navigator

Tags
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A 76-year-old retired steel industry employee is diagnosed with
renal cell carcinoma.
Answer Statistics
It is noted that his full blood count is grossly abnormal:

Haemoglobin (Hb) 203 g/L (130-180)


Test Analysis

Haematocrit (PCV) 0.60% (0.40-0.52)

Mean cell volume (MCV) 90 fL (84-96)

White cell count (WBC) 10 ×109/L (4.0-11.0)

Platelets 400 ×109/L (150-450)

Which of the following symptoms can be explained by the above


blood test results?

(Please select 1 option)


Onycholysis
Incorrect answer selected

Pruritus
This is the correct answer


Pyrexia


Torticollis
Score:

Vitiligo
Total Answered:

The above demonstrates a case of polycythaemia likely secondary


to the renal cell carcinoma. Feedback
Polycythaemia can result in symptoms of the hyperviscosity
syndrome, including:
dizziness Question Navigator
tinnitus
headaches Revision Notes
blurred vision, and
pruritus.
Tags
A classic symptom of polycythemia vera is pruritus or itching,
particularly after exposure to warm water (such as
when taking a
bath), which may be due to abnormal histamine release1
or prostaglandin production2.

Signs include:
Various ophthalmological changes (for example, dilated retinal
veins)
Neurological findings, and
Facial plethora ('ruddy' appearance).

Polycythaemia can be categorised into primary or secondary.

Primary is due to polycythaemia rubra vera (PRV), which is a


chronic myeloproliferative disorder often presenting
with a raised
haemoglobin, leukocytosis and thrombocytosis. Previously a raised
leukocyte alkaline
phosphatase was tested for, but this is now less
commonly performed. PRV is due to a genetic defect (JAK-2

mutation).

Secondary causes of polycythaemia include


erythropoietin-secreting tumours and causes of chronic hypoxia

Erythropoietin-secreting tumours:
Renal cell carcinoma
Hepatocellular carcinoma
Haemangioblastoma
Uterine fibroids.

Chronic hypoxia:
COPD
Right-to-left cardiac shunts
Sleep apnoea
High altitude
Chronic carbon monoxide poisoning (including heavy
smoking).

Reference:
1. Steinman HK, et al. Polycythaemia rubra vera and water-induced
pruritus: blood histamine levels and
cutaneous fibrinolytic
activity before and after water challenge. Br J
Dermatol. 1987;116(3):329-33.
2. Fjellner B, Hägermark O. Pruritus in polycythemia vera: treatment with
aspirin and possibility of platelet
involvement. Acta Derm
Venereol. 1979;59(6):505-12.

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A 34-year-old man with normal baseline cardiac and respiratory
function starts on the ABVD (Adriamycin,
Test Analysis
Bleomycin, Vinblastine and
Dacarbazine) chemotherapy regimen for his stage IIB Hodgkin's
lymphoma. He
tolerated the first three cycles of the chemotherapy
well.

After completion of the 4th cycle, he presents with exertional


dyspnoea and a dry cough. He is afebrile, a chest x
ray and ECG are
normal.

What is the most likely diagnostic possibility?

(Please select 1 option)



Adriamycin related cardiomyopathy


Bleomycin related pulmonary fibrosis


Hyperemesis and reflex cough related todacarbazine


Pneumocystis jirovecii pneumonia


Vinblastine related neurotoxicity
Score:

Total Answered:

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A 34-year-old man with normal baseline cardiac and respiratory
function starts on the ABVD (Adriamycin,
Answer Statistics
Bleomycin, Vinblastine and
Dacarbazine) chemotherapy regimen for his stage IIB Hodgkin's
lymphoma. He
tolerated the first three cycles of the chemotherapy
well.
Test Analysis
After completion of the 4th cycle, he presents with exertional
dyspnoea and a dry cough. He is afebrile, a chest x
ray and ECG are
normal.

What is the most likely diagnostic possibility?

(Please select 1 option)


Adriamycin related cardiomyopathy
Incorrect answer selected

Bleomycin related pulmonary fibrosis


This is the correct answer


Hyperemesis and reflex cough related todacarbazine


Pneumocystis jirovecii pneumonia


Vinblastine related neurotoxicity

Bleomycin related pulmonary fibrosis is a major toxicity of the


widely used ABVD regimen for treatment of
Score:
Hodgkins lymphoma. A high
resolution CT scan and pulmonary function tests are required to
diagnose this
condition. Total Answered:

Although doxorubicin (also known as adriamycin) can cause


cardiotoxicity, this is unusual at the doses used in
this regimen
and one would expect abnormalities on the ECG. Feedback

He is afebrile, so Pneumocystis jirovecii is less likely though


needs to be considered in the differential.
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There is no history of hyperemesis given and a vinblastine
neuropathy is unlikely to present as acute shortness
of breath.
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A mild lymphocytosis of 15 ×109/l is reported on a
full blood count result in a 70-year-old asymptomatic man
Test Analysis
attending
clinic for an annual review.

What would be the most essential investigation to establish a


diagnosis of chronic lymphocytic leukemia (CLL)?

(Please select 1 option)



CT scan of chest abdomen and pelvis


Lactic Dehydrogenase (LDH) levels


Peripheral blood flow cytometry


Presence of palpable cervical lymphadenopathy


Presence of smear cells on the blood film

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Score:

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A mild lymphocytosis of 15 ×109/l is reported on a
full blood count result in a 70-year-old asymptomatic man
Answer Statistics
attending
clinic for an annual review.

What would be the most essential investigation to establish a


diagnosis of chronic lymphocytic leukemia (CLL)? Test Analysis
(Please select 1 option)
CT scan of chest abdomen and pelvis
Incorrect answer selected


Lactic Dehydrogenase (LDH) levels

Peripheral blood flow cytometry


This is the correct answer


Presence of palpable cervical lymphadenopathy


Presence of smear cells on the blood film

Flow cytometry showing a specific pattern of monoclonal B cell


proliferation (CD19/5 co expressing, CD23
positive, light chain
restricted B cell population) is diagnostic of CLL.

CT scan and LDH are investigations needed to complete staging


but not essential for diagnosis. Smear cells are
reported in other
lymphoproliferative as well as benign lymphocytosis and merely
indicate fragile lymphocytes
Score:
which are artefactually smeared on the
glass slide.
Total Answered:
Cervical lymphadenopathy may be seen in CLL but can also be seen
in any other cause of lymphadenopathy
(e.g. viral infections,
adenopathy secondary to local dental infection).
Feedback

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A 64-year-old man presents with anaemia and blurring of vision
with a haemoglobin level of 89 g/L. A total
Test Analysis
protein with a raised
globulin fraction is noted.

A serum EPG shows an IgM paraprotein of 37 g/L. A bone marrow


aspirate shows a lymphoplasmacytic infiltrate.

Which of the following would be the next most important


investigation?

(Please select 1 option)



CT scan of head and orbits


Plasma viscosity


PET scan


Skeletal survey


Urine electrophoresis

Score:
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Total Answered:

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A 64-year-old man presents with anaemia and blurring of vision
with a haemoglobin level of 89 g/L. A total
Answer Statistics
protein with a raised
globulin fraction is noted.

A serum EPG shows an IgM paraprotein of 37 g/L. A bone marrow


aspirate shows a lymphoplasmacytic infiltrate. Test Analysis
Which of the following would be the next most important
investigation?

(Please select 1 option)


CT scan of head and orbits
Incorrect answer selected

Plasma viscosity
This is the correct answer


PET scan


Skeletal survey


Urine electrophoresis

This man has a lymphoplasmacytic lymphoma with an IgM


paraprotein which is diagnostic of Waldenstrom's

macroglobulinaemia.

One of the serious complications of this condition is


hyperviscosity syndrome. A plasma viscosity measurement Score:
would be
essential to diagnose and initiate treatment. The initial treatment
would be plasmapheresis followed by
Total Answered:
cytoreductive therapy.

A skeletal survey, CT scan and PET scan are not particularly


useful for diagnosis or prognosis of this condition.
Feedback
A urine electrophoresis is a investigation that is necessary but
not diagnostic in this situation where a diagnosis
of
hyperviscosity needs to be made. Question Navigator

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A 23-year-old female presents with rapid distension of her
abdomen. A biopsy from a abdominal mass shows a B
Test Analysis
cell
lymphoblastic lymphoma with a high proliferative index.

Which of the following is the cytogenetic abnormality seen in


this lymphoma?

(Please select 1 option)



t(4;11)


t(8;14)


t(11;18)


t(11;14)


t(14;18)

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A 23-year-old female presents with rapid distension of her
abdomen. A biopsy from a abdominal mass shows a B
Answer Statistics
cell
lymphoblastic lymphoma with a high proliferative index.

Which of the following is the cytogenetic abnormality seen in


this lymphoma? Test Analysis
(Please select 1 option)
t(4;11)
Incorrect answer selected

t(8;14)
This is the correct answer


t(11;18)


t(11;14)


t(14;18)

This lady has a high grade lymphoma which is commonly referred


to as Burkitt's lymphoma.

The characteristic cytogenetic abnormality is a reciprocal


translocation t(8;14) while t( 11;14) (equivalent to cyclin
D1) is
seen in mantle cell lymphoma and t(14;18) is seen in cases of
follicular lymphoma.

Burkitt's lymphoma is treated with intensive chemotherapy.


 It has high response rates and is a haematological Score:
diagnosis which
must be reached as an emergency in view of the high cell
turnover.
Total Answered:

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A 42-year-old male is investigated for dyspepsia. An antral
biopsy from an irregular area visualised in the
Test Analysis
gastroscopy is
reported as mucosal associated lymphoid tissue lymphoma (MALT
lymphoma).

He has no other sites of disease on staging and is systemically


well.

Which of the following would be the first line treatment of


choice for this man?

(Please select 1 option)



Chemotherapy in combination with monoclonal antibodies


Monoclonal antibody therapy


Radiotherapy


Surgery


Triple therapy

Score:
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A 42-year-old male is investigated for dyspepsia. An antral
biopsy from an irregular area visualised in the
Answer Statistics
gastroscopy is
reported as mucosal associated lymphoid tissue lymphoma (MALT
lymphoma).

He has no other sites of disease on staging and is systemically


well. Test Analysis
Which of the following would be the first line treatment of
choice for this man?

(Please select 1 option)


Chemotherapy in combination with monoclonal antibodies
Incorrect answer selected


Monoclonal antibody therapy


Radiotherapy


Surgery

Triple therapy
This is the correct answer

This man has Helicobacter pylori associated MALT


lymphoma. The first line therapy for this is using triple
therapy
(a common regimen is a proton pump inhibitor, amoxicillin and
clarithromycin). This usually results in
resolution of the lymphoma
in up to 80% of the cases.
Score:
Refractory or more widespread cases can be treated with systemic
chemotherapy sometimes in combination
Total Answered:
with radiotherapy.

Monoclonal antibodies are now routinely used for treatment of B


cell lymphomas but usually not as monotherapy.
Feedback
Surgery is generally not a treatment option for lymphoma.
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A 74-year-old man presents with severe constipation and
abdominal pains after the first cycle of his
Test Analysis
chemotherapy with
R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and
prednisolone).

Which of the following drugs would be implicated?

(Please select 1 option)



Cyclophosphamide


Doxorubicin


Prednisolone


Rituximab


Vincristine

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Total Answered:

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A 74-year-old man presents with severe constipation and
abdominal pains after the first cycle of his
Answer Statistics
chemotherapy with
R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine and
prednisolone).

Which of the following drugs would be implicated? Test Analysis


(Please select 1 option)
Cyclophosphamide
Incorrect answer selected


Doxorubicin


Prednisolone


Rituximab

Vincristine
This is the correct answer

Constipation is a common side effect of vincristine, a vinca


alkaloid. This is common in the elderly, where this
drug should be
used with caution.

Cyclophosphamide can cause urothelial mucosal toxicity.

Anthracyclines can cause cardiotoxicity and assessment of


cardiac function with echocardiography is important Score:
prior to using
this class of drug.
Total Answered:
Rituximab is a monoclonal antibody to CD20 which is usually
associated with infusional toxicity such as
hypotension and may
cause delayed neutropenia.
Feedback

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A 67-year-old man presents with symptomatic anaemia. On
examination he is jaundiced and has generalised
Test Analysis
lymphadenopathy
with moderate hepatosplenomegaly.

His investigations are given below:

Haemoglobin 81 g/L (135 - 180)

Mean corpuscular volume 108 fL (80 - 100)

White cell count 14.6 ×109/L (4 - 11)

Neutrophils 3.2 ×109/L (1.5 - 7.4)

Lymphocytes 7.0 ×109/L (1.1 - 4.0)

Bilirubin 34 mg/L (1-22)

LDH 560 IU/L -

Direct Coombs' test positive with IgG

Blood smear: Spherocytosis, lymphocytosis and smear cells. Score:

Which of the following is the most likely cause of his


anaemia? Total Answered:

(Please select 1 option)


Question Navigator

Acute lymphoblastic leukaemia with bone marrow infiltration


Chronic lymphocytic leukaemia (CLL) with bone marrow infiltration
Tags

Chronic lymphocytic leukaemia with autoimmune haemolysis


Diffuse large B cell lymphoma with autoimmune haemolysis


Hereditary spherocytosis

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A 67-year-old man presents with symptomatic anaemia. On
examination he is jaundiced and has generalised
Answer Statistics
lymphadenopathy
with moderate hepatosplenomegaly.

His investigations are given below: Test Analysis


Haemoglobin 81 g/L (135 - 180)

Mean corpuscular volume 108 fL (80 - 100)

White cell count 14.6 ×109/L (4 - 11)

Neutrophils 3.2 ×109/L (1.5 - 7.4)

Lymphocytes 7.0 ×109/L (1.1 - 4.0)

Bilirubin 34 mg/L (1-22)

LDH 560 IU/L -

Direct Coombs' test positive with IgG

Blood smear: Spherocytosis, lymphocytosis and smear cells.

Which of the following is the most likely cause of his


anaemia?
Score:
(Please select 1 option)
Total Answered:
Acute lymphoblastic leukaemia with bone marrow infiltration
Incorrect answer selected


Chronic lymphocytic leukaemia (CLL) with bone marrow infiltration
Feedback
Chronic lymphocytic leukaemia with autoimmune haemolysis
This is the correct answer


Diffuse large B cell lymphoma with autoimmune haemolysis Question Navigator


Hereditary spherocytosis
Revision Notes

About 10% of patients with CLL present with autoimmune


haemolysis. Tags
In this patient the combination of spherocytes with a raised
bilirubin, LDH and positive direct Coombs' test is
consistent
with an autoimmune haemolysis. The presence of generalised
lymphadenopathy with lymphocytosis
and smear cells suggests a
diagnosis of chronic lymphocytic leukaemia.

Although bone marrow infiltration with CLL can cause anaemia


(with reticulocytopenia) this would not be
accompanied by
biochemical markers of haemolysis.

An acute lymphoblastic leukaemia is less common in this age


group and the patient likely to have circulating
blasts in the
blood film rather than lymphocytosis.

Diffuse large B cell lymphoma does not usually cause haemolysis


while generalised lymphadenopathy is difficult
to explain in
hereditary spherocytosis.

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A 36-year-old man presents with stage IV diffuse large B cell
lymphoma. On the CT scan he has bulky
Test Analysis
abdominal nodes. He receives
combination chemotherapy along with rituximab.

About 24 hours later he reports feeling unwell and his


investigations are shown below:

Serum sodium 137 mmol/L (135-146)

Serum potassium 5.6 mmol/L (3.5-5.0)

Calcium 1.9 mmol/L (2.2-2.6)

Phosphate 1.42 mmol/L (0.8-1.4)

Urea 9.1 mmol/L (10-20)

Uric acid 1.2 mmol/L -

Creatinine 156 μmol/L (79-118)

Lactic acid dehydrogenase 1845 IU/L (105 - 333)

What is the diagnosis?


Score:
(Please select 1 option)
Total Answered:

Cyclophosphamide related urothelial toxicity


Doxorubicin related renal toxicity
Question Navigator

Infusional toxicity due to rituximab


Sepsis Tags

Tumour lysis syndrome

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A 36-year-old man presents with stage IV diffuse large B cell
lymphoma. On the CT scan he has bulky
Answer Statistics
abdominal nodes. He receives
combination chemotherapy along with rituximab.

About 24 hours later he reports feeling unwell and his


investigations are shown below: Test Analysis
Serum sodium 137 mmol/L (135-146)

Serum potassium 5.6 mmol/L (3.5-5.0)

Calcium 1.9 mmol/L (2.2-2.6)

Phosphate 1.42 mmol/L (0.8-1.4)

Urea 9.1 mmol/L (10-20)

Uric acid 1.2 mmol/L -

Creatinine 156 μmol/L (79-118)

Lactic acid dehydrogenase 1845 IU/L (105 - 333)

What is the diagnosis?

(Please select 1 option)


Cyclophosphamide related urothelial toxicity
Incorrect answer selected Score:

Doxorubicin related renal toxicity Total Answered:

Infusional toxicity due to rituximab


Sepsis Feedback
Tumour lysis syndrome
This is the correct answer
Question Navigator

This patient has tumour lysis syndrome evidenced by


hyperkalaemia, hyperphosphataemia, hypocalcaemia, Revision Notes
hyperuricaemia
and acute renal failure. It results from release of toxic
intracellular substances which occurs
during breakdown of rapidly
dividing tumours.
Tags
Sepsis or infusional toxicity of rituximab is not accompanied by
this metabolic profile.

Doxorubicin does not cause renal toxicity and cyclophosphamide


related urothelial toxicity often results in
haematuria.

Patients at high risk of tumour lysis syndrome should be managed


with aggressive hydration with 4-6 L per day,
and commencement of
rasburicase and/or allopurinol prior to commencing cytotoxic
therapy.

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A 27-year-old female with Hodgkin's lymphoma with a bulky
mediastinal mass at presentation has completed
Test Analysis
treatment with
combination chemotherapy and radiotherapy to the mediastinum.

For which of the following will this patient need long term
monitoring?

(Please select 1 option)



Bone marrow failure


Cataracts


Infertility


Osteoporosis


Secondary malignancy

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A 27-year-old female with Hodgkin's lymphoma with a bulky
mediastinal mass at presentation has completed
Answer Statistics
treatment with
combination chemotherapy and radiotherapy to the mediastinum.

For which of the following will this patient need long term
monitoring? Test Analysis
(Please select 1 option)
Bone marrow failure
Incorrect answer selected


Cataracts


Infertility


Osteoporosis

Secondary malignancy
This is the correct answer

Secondary malignancy is the long term complication of highest


concern of the treatment this lady has received.
This patient would
also be at increased risk of breast cancer given the radiotherapy
to the mediastinum that she
has received.

Bone marrow failure is unusual in the setting of Hodgkin's


lymphoma unless the patient has had multiple salvage
Score:
regimens as
treatment. Fertility is usually preserved unless there has been
gonadal irradiation. Cataracts and
osteoporosis are not specific to
the type of treatment described in this lady. Total Answered:

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A 41-year-old lady is diagnosed with stage IV non-bulky
follicular lymphoma (low grade).
Test Analysis
She is asymptomatic apart from a few small neck nodes which her
GP noticed on routine examination. Her full
blood count, renal
function and LDH are normal.

Which of the following would be the next appropriate management


plan?

(Please select 1 option)



Allogeneic bone marrow transplant


Autologous bone marrow transplant


Imunosuppressive therapy


Intensive chemotherapy


Wait and watch approach

Score:
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A 41-year-old lady is diagnosed with stage IV non-bulky
follicular lymphoma (low grade).
Answer Statistics
She is asymptomatic apart from a few small neck nodes which her
GP noticed on routine examination. Her full
blood count, renal
function and LDH are normal. Test Analysis
Which of the following would be the next appropriate management
plan?

(Please select 1 option)


Allogeneic bone marrow transplant
Incorrect answer selected


Autologous bone marrow transplant


Imunosuppressive therapy


Intensive chemotherapy

Wait and watch approach


This is the correct answer

Asymptomatic patients with low grade lymphoma such as follicular


lymphoma (grade 1 and 2) can be observed
closely.

The value of intensive chemotherapy is questionable in


asymptomatic patients. No long term survival benefit has Score:
been
demonstrated with this approach.
Total Answered:
An autologous or an allogeneic transplant would not be
considered front line therapy for lymphomas.

Immunosuppressive therapy as a monotherapy is seldom employed as


treatment in lymphomas. Feedback
Enrollment in a clinical trial would be an equally acceptable
option in this case as best treatment approach is yet
to be
established. If this patient were symptomatic or had end organ
damage due to lymphoma she would have Question Navigator
needed to start
treatment.
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A 18-year-old gentleman with T-cell acute lymphoblastic
leukaemia has been administered cytotoxic
Test Analysis
chemotherapy with
cyclophosphamide, doxorubicin, vincristine and corticosteroids
(hyper-CVAD regimen).

On day 14 from starting chemotherapy the patient is noted to


have a BP of 100/60 mmHg, a pulse rate of 120
bpm, a fever of 39°C
with chills and rigors.

Which of the following will be your first intervention in this


case?

(Please select 1 option)



Aggressive resuscitation with colloid fluids


Blood and urine cultures and chest radiograph, together with
C-reactive protein


Consideration for more intensive support on ITU


Initiation of inotrope support


Prompt administration of broad-spectrum antimicrobials to cover
Gram negative and Gram positive organisms within
minutes

Score:

Total Answered:
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A 18-year-old gentleman with T-cell acute lymphoblastic
leukaemia has been administered cytotoxic
Answer Statistics
chemotherapy with
cyclophosphamide, doxorubicin, vincristine and corticosteroids
(hyper-CVAD regimen).

On day 14 from starting chemotherapy the patient is noted to


have a BP of 100/60 mmHg, a pulse rate of 120 Test Analysis
bpm, a fever of 39°C
with chills and rigors.

Which of the following will be your first intervention in this


case?

(Please select 1 option)


Aggressive resuscitation with colloid fluids
Incorrect answer selected


Blood and urine cultures and chest radiograph, together with
C-reactive protein


Consideration for more intensive support on ITU


Initiation of inotrope support

Prompt administration of broad-spectrum antimicrobials to cover


Gram negative and Gram positive organisms within
minutes
This is the correct answer

This question covers the important topic of management of


neutropenic sepsis.
Score:
Albeit management with fluids, inotropes, culturing to discover
the entity of the pathogen in every case are all
important, the
single most important intervention in neutropenic sepsis is the
prompt institution of antimicrobial Total Answered:
therapy, with broad-spectrum
antimicrobials, for example, piperacillin/tazobactam and
gentamicin.

Reference: Feedback

NICE. Neutropenic sepsis (CG151).


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A 25-year-old gentleman with Burkitt's lymphoma is admitted
and commenced on induction chemotherapy.
Test Analysis
Within 48 hours it is noticed that his urine output is dropping
to 20 mls/hr.

Further investigation shows:

Potassium 6.5 mmol/L (3.5-4.9)

Calcium 1.5 mmol/L (2.2-2.6)

Phosphate 4 mmol/L (0.8-1.4)

Creatinine 250 µmol/L (60-110)

Which of the following is the most appropriate management of


this complication?

(Please select 1 option)



Allopurinol and intravenous hydration


Intravenous hydration with 3 L/m2 and rasburicase

Score:

Intravenous hydration with 3 litres per day
Total Answered:

Rasburicase only


Urinary alkalinisation
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A 25-year-old gentleman with Burkitt's lymphoma is admitted
and commenced on induction chemotherapy.
Answer Statistics
Within 48 hours it is noticed that his urine output is dropping
to 20 mls/hr.

Further investigation shows: Test Analysis

Potassium 6.5 mmol/L (3.5-4.9)

Calcium 1.5 mmol/L (2.2-2.6)

Phosphate 4 mmol/L (0.8-1.4)

Creatinine 250 µmol/L (60-110)

Which of the following is the most appropriate management of


this complication?

(Please select 1 option)


Allopurinol and intravenous hydration
Incorrect answer selected

Intravenous hydration with 3 L/m2 and rasburicase


This is the correct answer


Intravenous hydration with 3 litres per day


Rasburicase only
Score:

Urinary alkalinisation
Total Answered:

This question concerns the management of acute tumour lysis


syndrome (ATLS). Feedback
Acute tumour lysis syndrome is a common complication of
haematological tumours which have a high
proliferation index, for
example, Burkitt's lymphoma, hyperleukocytic acute myelogenous
leukaemia (AML), Question Navigator
diffuse large B-cell lymphoma.

Management revolves around institution of aggressive hydration,


aiming for 3 L/m2 control of electrolyte Revision Notes
disturbances
(typically, hypocalcaemia, hyperphosphataemia, hyperkalaemia and
uraemia) and clearance of the
increased metabolic load with
rasburicase, a specific recombinant enzyme. Tags
The incorrect answer options omit important aspects of the
holistic management of acute tumour lysis syndrome.

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A 10-year-old boy is noticed to be jaundiced on return from a
holiday in Africa with his parents. He is on
Test Analysis
antimalarial
prophylaxis.

His complete blood count shows haemoglobin of 80 g/L, with Heinz


bodies and blister cells on blood film
examination.

Which of the following relates to this disorder?

(Please select 1 option)



It is most commonly precipitated by peas


The antimalarial prophylaxis has no relation to the laboratory
findings


This is a common autosomal dominant disorder


Transfusion is strictly merited in each case


Treatment involves strict avoidance of known precipitants

Score:

Total Answered:
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A 10-year-old boy is noticed to be jaundiced on return from a
holiday in Africa with his parents. He is on
Answer Statistics
antimalarial
prophylaxis.

His complete blood count shows haemoglobin of 80 g/L, with Heinz


bodies and blister cells on blood film Test Analysis
examination.

Which of the following relates to this disorder?

(Please select 1 option)


It is most commonly precipitated by peas
Incorrect answer selected


The antimalarial prophylaxis has no relation to the laboratory
findings


This is a common autosomal dominant disorder


Transfusion is strictly merited in each case

Treatment involves strict avoidance of known precipitants


This is the correct answer

This question relates to the management of G-6-PD


deficiency.

G-6-PD is the most common inherited enzyme defect in humans; an


X linked inheritance pattern. Score:

It is mostly commonly precipitated by several classes of drugs,


including antimalarials and is also associated with Total Answered:
ingestion of
broad beans (favism).

Treatment revolves around avoidance of all known precipitating


factors, and blood product support should only Feedback
be given in
unstable, symptomatic patients.
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A 7-month-old baby of Afro-American descent is noticed to have
swollen fingers.
Test Analysis
His blood count shows haemoglobin of 72 g/L and haemoglobin
electrophoresis shows a haemoglobin S level of
98%.

Which of the following relates to this haematological


disorder?

(Please select 1 option)



Aplastic crises are most usually caused by Salmonella


Most patients should be routinely transfused at each episode


Most patients require a haemopoietic stem cell transplant


Painful crises can usually be managed with simple analgesia and
hydration only


Therapies directed towards increasing the fraction of fetal
haemoglobin have made a difference, eg. hydroxycarbamide

Score:
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A 7-month-old baby of Afro-American descent is noticed to have
swollen fingers.
Answer Statistics
His blood count shows haemoglobin of 72 g/L and haemoglobin
electrophoresis shows a haemoglobin S level of
98%. Test Analysis
Which of the following relates to this haematological
disorder?

(Please select 1 option)


Aplastic crises are most usually caused by Salmonella
Incorrect answer selected


Most patients should be routinely transfused at each episode


Most patients require a haemopoietic stem cell transplant


Painful crises can usually be managed with simple analgesia and
hydration only

Therapies directed towards increasing the fraction of fetal


haemoglobin have made a difference, eg.
hydroxycarbamide
This is the correct answer

Sickle cell anaemia requires a multifaceted approach to


management. It is important to enquire about each
patient's
baseline haemoglobin since most patients do not require blood
product support at each presentation.
Score:
The most frequent complications include painful (usually
requiring opioid analgesia), aplastic (most frequently
caused by
parvovirus B19 infection; Salmonella tends to cause
osteomyelitis) and sequestration crises. Total Answered:

Although haemopoietic stem cell transplant can be curative, most


patients do not require it in view of the difficulty
in
anticipating whose clinical course will be severe enough when
assessed during childhood. Feedback

Therapy which improves the fraction of fetal haemoglobin by even


2-4% is enough to ameliorate the clinical
picture considerably.
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A 35-year-old gentleman diagnosed with acute lymphoblastic
leukaemia underwent a matched sibling allogeneic
Test Analysis
haemopoietic stem
cell transplant.

One month after the transplant the patient developed profuse


haemoptysis, which persists despite platelet
support. He also
developed low grade pyrexia.

You would proceed to do which of the following?

(Please select 1 option)



Investigate with serological evidence of fungal infection -
galactomannan or D-glucan testing and/or PCR for fungal
DNA


Investigate with imaging of the chest


Proceed to do a bronchoscopy and obtain BAL for further
investigation


Start empirical treatment with liposomal amphotericin B


Start empirical treatment with caspofungin

Score:

Total Answered:
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A 35-year-old gentleman diagnosed with acute lymphoblastic
leukaemia underwent a matched sibling allogeneic
Answer Statistics
haemopoietic stem
cell transplant.

One month after the transplant the patient developed profuse


haemoptysis, which persists despite platelet Test Analysis
support. He also
developed low grade pyrexia.

You would proceed to do which of the following?

(Please select 1 option)


Investigate with serological evidence of fungal infection -
galactomannan or D-glucan testing and/or PCR for fungal
DNA
Incorrect answer selected

Investigate with imaging of the chest


This is the correct answer


Proceed to do a bronchoscopy and obtain BAL for further
investigation


Start empirical treatment with liposomal amphotericin B


Start empirical treatment with caspofungin

This question covers the use of antifungals in haematology


patients.
Score:
In patients who are prone to invasive fungal infections it is no
longer suggested that empirical therapy with
systemic antifungals
is commenced (according to the latest British Committee for
Standards in Haematology Total Answered:
guidelines).

Serological and/or imaging/biopsy should be sought prior to


instituting antifungal therapy, but imaging would be Feedback
the least
invasive and most easily available modality of choice in order to
investigate a suspected fungal
infection. Question Navigator

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Which of the following observations is consistent with the red
cell membrane disorder hereditary elliptocytosis?
Test Analysis
(Please select 1 option)

Heterozygotes are usually asymptomatic


It is caused by vertical red cell membrane, that is, ankyrin
spectrin link defect


The red cells haemolyse on exposure to drugs


There are no specific red cell morphology features


Splenectomy is advisable in most patients

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Which of the following observations is consistent with the red
cell membrane disorder hereditary elliptocytosis?
Answer Statistics
(Please select 1 option)
Heterozygotes are usually asymptomatic
Correct Test Analysis

It is caused by vertical red cell membrane, that is, ankyrin
spectrin link defect


The red cells haemolyse on exposure to drugs


There are no specific red cell morphology features


Splenectomy is advisable in most patients

Red cell membrane defects are broadly classified into hereditary


spherocytosis (HS), hereditary elliptocytosis
(HE) and others.

Horizontal membrane protein defects (for example, spectrin


ankyrin interaction defect) results in HE whereas
vertical defects
result in hereditary spherocytosis.

Elliptocytosis is usually caused by spectrin and


spectrin-protein 4.1 defects.

Heterozygotes are asymptomatic but show elliptocytes on blood


film; they do not have haemolysis and do not Score:
require any particular
treatment (hence excluding other options).
Total Answered:

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A 14-month-old baby is admitted with an Hb of 58 g/L and a
diagnosis of Fanconi's anaemia is suspected.
Test Analysis
Which of the following observations would be consistent with the
pathology in this condition?

(Please select 1 option)



Haemoglobin synthesis disorder


Increased chromosomal fragility disorder


Increased extravascular red cell destruction


Increased intravascular red cell destruction


Iron metabolism disorder

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A 14-month-old baby is admitted with an Hb of 58 g/L and a
diagnosis of Fanconi's anaemia is suspected.
Answer Statistics
Which of the following observations would be consistent with the
pathology in this condition?

(Please select 1 option) Test Analysis


Haemoglobin synthesis disorder
Incorrect answer selected

Increased chromosomal fragility disorder


This is the correct answer


Increased extravascular red cell destruction


Increased intravascular red cell destruction


Iron metabolism disorder

Fanconi's anaemia (FA) is characterised by pancytopenia,


infections and transfusion dependence occurring in
childhood.

A typical feature of cells from patients with FA is


hypersensitivity to genetic damage induced by DNA-damaging
and
cross-linking agents such as diethyl benzene (DEB), mitomycin C
(MMC), and ionising radiation (thus being
synonymous with a
chromosomal breakage or fragility disorder).
Score:
The condition is a congenital bone marrow failure syndrome
relating to mutations in DNA repair mechanisms.
Total Answered:

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A 52-year-old man develops a deep vein thrombosis after being
admitted for a fracture of his left femur following
Test Analysis
a car
crash.

Which of the following is true about thrombophilia testing?

(Please select 1 option)



A thrombosis in an unusual vascular territory warrants
thrombophilia testing


All patients, irrespective of a family history of thrombosis,
require to be tested


Patients with provoked DVT should always be tested for
thrombophilia


The most common known heritable thrombophilia is prothrombin gene
mutation


This patient warrants testing as he is less than 60 years of age

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Score:

Total Answered:

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A 52-year-old man develops a deep vein thrombosis after being
admitted for a fracture of his left femur following
Answer Statistics
a car
crash.

Which of the following is true about thrombophilia testing? Test Analysis


(Please select 1 option)
A thrombosis in an unusual vascular territory warrants
thrombophilia testing
Correct


All patients, irrespective of a family history of thrombosis,
require to be tested


Patients with provoked DVT should always be tested for
thrombophilia


The most common known heritable thrombophilia is prothrombin gene
mutation


This patient warrants testing as he is less than 60 years of age

Thrombophilia testing is considered useful in patients


presenting with:
A first episode of venous thromboembolism (VTE) at a young age
(usually considered less than 45 years
of age)
Idiopathic venous thrombosis (this patient clearly has a
provoked DVT relating to a fracture)
A family history of thrombosis, particularly in a first degree
relative Score:
VTE in an unusual vascular territory
Total Answered:
Neonatal purpura fulminans
Warfarin induced skin necrosis

Factor V Leiden is the commonest heritable thrombophilia, a


heterozygote state is seen in about 5% of the
Feedback
general Caucasian
population.
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A 32-year-old woman is admitted with acute cholecystitis.
Imaging shows she has gallstones.
Test Analysis
Full blood count shows an Haemoglobin 104 g/L, reticulocytosis
and a blood film showing numerous
spherocytes. The direct
Coombs' (DAT) test is negative.

On further enquiry the lady gives a history of her mother having


a splenectomy for her anaemia.

What is the likely diagnosis?

(Please select 1 option)



Autoimmune haemolytic anaemia


Hereditary elliptocytosis


Hereditary spherocytosis


Sickle cell anaemia


Thalassaemia

Score:

Total Answered:
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A 32-year-old woman is admitted with acute cholecystitis.
Imaging shows she has gallstones.
Answer Statistics
Full blood count shows an Haemoglobin 104 g/L, reticulocytosis
and a blood film showing numerous
spherocytes. The direct
Coombs' (DAT) test is negative. Test Analysis
On further enquiry the lady gives a history of her mother having
a splenectomy for her anaemia.

What is the likely diagnosis?

(Please select 1 option)


Autoimmune haemolytic anaemia
Incorrect answer selected


Hereditary elliptocytosis

Hereditary spherocytosis
This is the correct answer


Sickle cell anaemia


Thalassaemia

Hereditary elliptocytosis (HE) heterozygote is usually


asymptomatic without haemolysis.
Score:
Thalassaemia and sickle cell anaemia can cause gallstones but
often have specific blood film changes. For
example, microcytic
hypochromic red cells in thalassaemia and sickle cells in sickle
cell anaemia respectively. Total Answered:

Autoimmune haemolysis would show a positive DAT.


Feedback
The history of anaemia in the family, with numerous spherocytes
and a negative DAT, suggests hereditary
spherocytosis as the cause
of anaemia in this case.
Question Navigator
A history of neonatal jaundice should also be sought.

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A 24-year-old male is admitted with severe fatigue.
Test Analysis
His full blood count shows:

Haemoglobin 45 g/L (130-180)

Neutrophils 0.7 ×109/L (1.5-7)

Platelets 14 ×109/L (150-400)

Reticulocyte count 1 ×109/L (25-85)

The bone marrow flow cytometry was reported as being non-


diagnostic. The bone marrow trephine was
reported as being
hypocellular with a few islands of erythropoiesis.

What is the most likely diagnosis?

(Please select 1 option)



Acute lymphoblastic
Score:

Acute myeloid leukaemia
Total Answered:

Aplastic anaemia


Haemolytic anaemia
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Myelodysplastic syndrome

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A 24-year-old male is admitted with severe fatigue.
Answer Statistics
His full blood count shows:

Haemoglobin 45 g/L (130-180)


Test Analysis

Neutrophils 0.7 ×109/L (1.5-7)

Platelets 14 ×109/L (150-400)

Reticulocyte count 1 ×109/L (25-85)

The bone marrow flow cytometry was reported as being non-


diagnostic. The bone marrow trephine was
reported as being
hypocellular with a few islands of erythropoiesis.

What is the most likely diagnosis?

(Please select 1 option)


Acute lymphoblastic
Incorrect answer selected


Acute myeloid leukaemia

Aplastic anaemia
This is the correct answer
Score:

Haemolytic anaemia
Total Answered:

Myelodysplastic syndrome

Feedback
This young man has a pancytopenia with a hypocellular marrow
therefore the diagnosis is most likely aplastic
anaemia. Question Navigator

Leukaemias are generally hypercellular and myelodysplastic


syndrome is unusual in this age group (but not
Revision Notes
unheard of,
especially if there is a previous history of ionising radiation or
alkylating agent use). Therefore the
answer is not acute
lymphoblastic, acute myeloid leukaemia or myelodysplastic
syndrome.
Tags
Haemolytic anaemias may cause pancytopenia in the setting of
hypersplenism, paroxysmal nocturnal
haemoglobinuria (PNH) or
aplastic crisis (due to erythrovirus infection) but there are
usually other clues or
indicators in those circumstances. Hence the
answer is not haemolytic anaemia.

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A 58-year-old female presents with a platelet count of 768
×109/L (150 - 400).
Test Analysis
On examination she has isolated mild splenomegaly. The history
and remainder of the examination are non-
contributory.

What would be the next most useful diagnostic test?

(Please select 1 option)



Bone marrow biopsy with molecular studies


Peripheral blood flow cytometry


PET scan


Splenic biopsy with immunohistochemistry


Staging CT scan

Score:
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A 58-year-old female presents with a platelet count of 768
×109/L (150 - 400).
Answer Statistics
On examination she has isolated mild splenomegaly. The history
and remainder of the examination are non-
contributory. Test Analysis
What would be the next most useful diagnostic test?

(Please select 1 option)


Bone marrow biopsy with molecular studies
Correct


Peripheral blood flow cytometry


PET scan


Splenic biopsy with immunohistochemistry


Staging CT scan

The presence of splenomegaly in the setting of a raised platelet


count suggests a diagnosis of a
myeloproliferative disorder.

A bone marrow biopsy with ancillary studies would be useful in


the diagnosis. Score:

Although not specific for any myeloproliferative disorder,


mutations of the Janus kinase 2 region are found in the Total Answered:
majority of
polycythaemia vera and in a significant proportion of essential
thrombocytosis. There are also certain
morphological features in
the megakaryocytes on the bone marrow trephine which would be
useful along with Feedback
assessment of marrow iron stores. Hence bone
marrow biopsy with molecular studies would be the next most
useful
diagnostic test.
Question Navigator
Radiology is not particularly useful and flow cytometry findings
are usually not specific in these disorders. Splenic
biopsy is
usually not undertaken for diagnosis of myeloproliferative
disorders.
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A 33-year-old man presents with headaches, blurring of vision
and a Haemoglobin of 210 g/L and a Haematocrit
Test Analysis
of 0.62.

The white cell and platelet counts are normal and there are no
diagnostic features on the blood film. He is a non-
smoker.

What is the next most important step?

(Please select 1 option)



Check erythropoietin levels


Organise for a renal ultrasound and a chest x ray


Perform Jak2 mutation status


Plasmapheresis


Venesection

Score:

Total Answered:
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A 33-year-old man presents with headaches, blurring of vision
and a Haemoglobin of 210 g/L and a Haematocrit
Answer Statistics
of 0.62.

The white cell and platelet counts are normal and there are no
diagnostic features on the blood film. He is a non- Test Analysis
smoker.

What is the next most important step?

(Please select 1 option)


Check erythropoietin levels
Incorrect answer selected


Organise for a renal ultrasound and a chest x ray


Perform Jak2 mutation status


Plasmapheresis

Venesection
This is the correct answer

Venesection of patients who are symptomatic is the first line


management of polycythaemia.

The diagnostic workup and exclusion of secondary causes usually


follows after initial treatment. This patient has Score:
symptoms of
hyperviscosity and needs to be venesected urgently and an agreed
work-up can be performed
Total Answered:
later.

This patient is at risk of thrombosis due to hyperviscosity and


needs treatment urgently.
Feedback

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A 45-year-old Caucasian lady presents with platelet count of 511
×109/L.
Test Analysis
She also has a microcytic hypochromic blood picture with a
haemoglobin of 102 g/L.

What is the next most important investigation?

(Please select 1 option)



Bone marrow biopsy with Jak2 mutation status


Check iron status with investigation for occult blood loss


Check peripheral blood for bcr-abl


PET scan to rule out a malignancy


Staging CT scan to rule out a lymphoma

Submit answer
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Total Answered:

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A 45-year-old Caucasian lady presents with platelet count of 511
×109/L.
Answer Statistics
She also has a microcytic hypochromic blood picture with a
haemoglobin of 102 g/L.

What is the next most important investigation? Test Analysis

(Please select 1 option)


Bone marrow biopsy with Jak2 mutation status
Incorrect answer selected

Check iron status with investigation for occult blood loss


This is the correct answer


Check peripheral blood for bcr-abl


PET scan to rule out a malignancy


Staging CT scan to rule out a lymphoma

Although a bone marrow biopsy or checking for bcr-abl mutation


may be useful to confirm a myeloproliferative
disorder, ruling out
iron deficiency as a cause for thrombocytosis is of paramount
importance in this patient.

A gain of function somatic mutation of Janus kinase gene JAK2


V617F has been associated with
myeloproliferative disorders. This
has been decribed in >95% of patients with polycythaemia vera
and in about Score:
40-50% of patients with essential thrombocytosis.
Total Answered:
A secondary cause for thrombocytosis is crucial to exclude
before considering a diagnosis of a myeloproliferative

disorder. Feedback
In fact, adequate iron stores are requisite diagnostic criteria
(WHO) for essential thrombocytosis.

Imaging is not useful unless a specific pathology is being


sought.
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A 19-year-old girl presents with a three week history of fever,
night sweats and weight loss.
Test Analysis
Physical examination shows hepatosplenomegaly. Her blood count
shows:

WBC 130 ×109/L (4-11)

Haemoglobin 68 g/L (115-165)

Platelets 21 ×109/L (150-400)

Peripheral smear shows circulating blasts and these are


confirmed to be B-ALL by flow cytometry
immunophenotyping.
Cytogenetics confirms positivity for BCR-ABL (Philadelphia
chromosome).

Commencement of which of the following is the most appropriate


therapy at this point?

(Please select 1 option)



Allogeneic stem cell transplant


Chemotherapy + rituximab + Tyrosine Kinase Inhibitor
Score:

High dose chemotherapy, e.g. UKALL 14, hyper-CVAD
Total Answered:

Rituximab


Tyrosine kinase inhibitor, e.g. dasatinib, nilotinib,imatinib
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A 19-year-old girl presents with a three week history of fever,
night sweats and weight loss.
Answer Statistics
Physical examination shows hepatosplenomegaly. Her blood count
shows:

130 ×109/L
Test Analysis
WBC (4-11)

Haemoglobin 68 g/L (115-165)

Platelets 21 ×109/L (150-400)

Peripheral smear shows circulating blasts and these are


confirmed to be B-ALL by flow cytometry
immunophenotyping.
Cytogenetics confirms positivity for BCR-ABL (Philadelphia
chromosome).

Commencement of which of the following is the most appropriate


therapy at this point?

(Please select 1 option)


Allogeneic stem cell transplant
Incorrect answer selected

Chemotherapy + rituximab + Tyrosine Kinase Inhibitor


This is the correct answer


High dose chemotherapy, e.g. UKALL 14, hyper-CVAD


Rituximab
Score:

Tyrosine kinase inhibitor, e.g. dasatinib, nilotinib,imatinib
Total Answered:

Philadelphia positive acute lymphoblastic leukaemia entails a


worse prognosis than BCR-ABL negative ALL. Feedback
The management must be tailored to hit both the acute leukaemia
with high dose chemotherapy, usually UKALL
14 or hyper-CVAD,
together with the anti-CD20 monoclonal antibody rituximab and a
tyrosine kinase inhibitor in Question Navigator
view of the BCR-ABL positivity.
Revision Notes
Hence chemotherapy + rituximab + Tyrosine Kinase Inhibitor (TKI)
is the correct answer.

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A 25-year-old lady with a history of nodular sclerosing Hodgkin
lymphoma in remission for nine months after six
Test Analysis
cycles of ABVD
chemotherapy presents again with a one month history of dry
cough. 

Chest radiograph shows mediastinal widening, and PET scanning


shows active thoracic disease.  Biopsy of the
mediastinal mass
reveals nodular sclerosing Hodgkin.

What is the most appropriate management option for this


lady?

(Please select 1 option)



Administer involved field radiotherapy


Autologous stem cell transplant only


Repeat ABVD chemotherapy


Salvage chemotherapy with ESHAP


Salvage chemotherapy followed by autologous stem cell transplant

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A 25-year-old lady with a history of nodular sclerosing Hodgkin
lymphoma in remission for nine months after six
Answer Statistics
cycles of ABVD
chemotherapy presents again with a one month history of dry
cough. 

Chest radiograph shows mediastinal widening, and PET scanning


shows active thoracic disease.  Biopsy of the Test Analysis
mediastinal mass
reveals nodular sclerosing Hodgkin.

What is the most appropriate management option for this


lady?

(Please select 1 option)


Administer involved field radiotherapy
Incorrect answer selected


Autologous stem cell transplant only


Repeat ABVD chemotherapy


Salvage chemotherapy with ESHAP

Salvage chemotherapy followed by autologous stem cell transplant


This is the correct answer

Relapsed Hodgkin lymphoma in an early relapse (less than one


year) must be managed aggressively with
salvage chemotherapy
followed by BEAM conditioned autologous stem cell transplantation
as the established
Score:
gold standard.
Total Answered:
Repeating ABVD chemotherapy is an option if this were a late
relapse.

IFRT has no role in the management of relapsed disease.


Feedback
Chemotherapy and ASCT have to be combined for maximal disease
clearing effect, so on their own they are
incorrect options. Question Navigator

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A 50-year-old gentleman presents with a three month history of
pruritus, headache and sweating. He does not
Test Analysis
smoke or have any
history of respiratory disease.

SpO2 is at 99% breathing room air.

His blood count shows:

WBC 6.3 ×109/L (4 - 11)

Hgb 18 g/dL (13-18)

Hct 58%

Platelet 511 ×109/L (150-400)

Which is the most useful diagnostic test for the diagnosis of


this myeloproliferative disorder?

(Please select 1 option)



BCR-ABL
Score:

JAK-2 mutation, including exon 12 mutations
Total Answered:

LDH


Serum erythropoietin
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Serum ferritin

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A 50-year-old gentleman presents with a three month history of
pruritus, headache and sweating. He does not
Answer Statistics
smoke or have any
history of respiratory disease.

SpO2 is at 99% breathing room air. Test Analysis


His blood count shows:

WBC 6.3 ×109/L (4 - 11)

Hgb 18 g/dL (13-18)

Hct 58%

Platelet 511 ×109/L (150-400)

Which is the most useful diagnostic test for the diagnosis of


this myeloproliferative disorder?

(Please select 1 option)


BCR-ABL
Incorrect answer selected

JAK-2 mutation, including exon 12 mutations


This is the correct answer


LDH
Score:

Serum erythropoietin
Total Answered:

Serum ferritin

Feedback
Polycythaemia rubra vera is a clonal myeloproliferative disorder
with increased myeloid, erythroid and
megakaryocyte precursor
production. Question Navigator
The diagnosis is based on exclusion of other myeloproliferative
disorders (namely CML via BCR-ABL), exclusion
of secondary causes
of polycythaemia with serum EPO level and normal oxygenation;
hence, positivity for the Revision Notes
JAK-2 mutation, which is found in almost
100% of cases of PRV, is diagnostic of the myeloproliferative
disorder.
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A 60-year-old lady with a history of diabetes mellitus,
essential hypertension and ischaemic heart disease with a
Test Analysis

myocardial infarct, is noted to have platelet count of 700 x


109/L (150-400).

Bone marrow biopsy shows increased megakaryocytes with lobulated


nuclei and abnormal localisation.  JAK-2
mutation is positive.

What is the correct management of this patient with essential


thrombocythaemia?

(Please select 1 option)



Anagrelide


Aspirin


Aspirin + hydroxycarbamide


Hydroxycarbamide


Interferon

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A 60-year-old lady with a history of diabetes mellitus,
essential hypertension and ischaemic heart disease with a
Answer Statistics

myocardial infarct, is noted to have platelet count of 700 x


109/L (150-400).

Bone marrow biopsy shows increased megakaryocytes with lobulated


nuclei and abnormal localisation.  JAK-2 Test Analysis
mutation is positive.

What is the correct management of this patient with essential


thrombocythaemia?

(Please select 1 option)


Anagrelide
Incorrect answer selected


Aspirin

Aspirin + hydroxycarbamide
This is the correct answer


Hydroxycarbamide


Interferon

Essential thrombocythaemia is a clonal myeloproliferative


disorder characterised by an elevated platelet count in
the absence
of a reactive cause for the thrombocytosis.
Score:
Fifty per cent of cases are positive for the JAK-2 mutation. 
Positivity for JAK-2 mutation, a platelet count above
Total Answered:
600 x
109/L and a past history of thrombosis entail a higher
risk, especially in the presence of other vascular risk
factors
such as hypertension, diabetes mellitus, ischaemic heart disease
and/or peripheral vascular disease.
Feedback
The management is based on cytoreduction with hydroxycarbamide
and an antiplatelet agent such as aspirin to
decrease the
thrombotic risk.
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Anagrelide is a second line drug in ET and has been associated
with increased bone marrow fibrosis.

Interferon has a use in ET in the setting of pregnancy. Revision Notes

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A 51-year-old gentleman newly diagnosed with myeloma is
commenced on treatment with dexamethasone and
Test Analysis
lenalidomide (an
immunomodulatory drug in the same class of drugs as
thalidomide).

Which treatment related issue MUST be discussed and clearly


documented in the notes?

(Please select 1 option)



Constipation


Myelosuppression


Peripheral neuropathy


Somnolence


Teratogenic potential

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A 51-year-old gentleman newly diagnosed with myeloma is
commenced on treatment with dexamethasone and
Answer Statistics
lenalidomide (an
immunomodulatory drug in the same class of drugs as
thalidomide).

Which treatment related issue MUST be discussed and clearly


documented in the notes? Test Analysis
(Please select 1 option)
Constipation
Incorrect answer selected


Myelosuppression


Peripheral neuropathy


Somnolence

Teratogenic potential
This is the correct answer

Immunomodulatory drugs such as thalidomide and lenalidomide are now first line medications in
the treatment of
myeloma. The most common side effect of
lenalidomide is myelosuppression, whereas somnolence, peripheral

neuropathy and constipation are side effects of thalidomide.

The inherent, serious issue that is applicable to both


medications is the teratogenic potential - all patients must
Score:
be
informed of this risk and advised regarding birth control and
avoidance of sharing of medications with any
other person. Total Answered:

It is not known whether lenalidomide is present in the semen of


male patients receiving the drug. Therefore,
males receiving
lenalidomide must always use a latex condom during any sexual
contact with females of Feedback
childbearing potential even if they have
undergone a successful vasectomy.
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A 32-year-old lady, 40 days post matched unrelated donor (MUD)
allogeneic bone marrow transplant in her
Test Analysis
second remission of her
acute myeloid leukaemia complains of loose bowel motions and a
maculopapular rash
over her extremities.

Her liver function tests showed an elevated bilirubin, alkaline


phosphatase, with sparing of ALT and GGT. The
patient is diagnosed
with acute graft versus host disease.

What is the most appropriate initial management?

(Please select 1 option)



Azathioprine


Methotrexate


Methylprednisone + cyclosporine


Mycophenolate mofetil


Tacrolimus
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A 32-year-old lady, 40 days post matched unrelated donor (MUD)
allogeneic bone marrow transplant in her
Answer Statistics
second remission of her
acute myeloid leukaemia complains of loose bowel motions and a
maculopapular rash
over her extremities.
Test Analysis
Her liver function tests showed an elevated bilirubin, alkaline
phosphatase, with sparing of ALT and GGT. The
patient is diagnosed
with acute graft versus host disease.

What is the most appropriate initial management?

(Please select 1 option)


Azathioprine
Incorrect answer selected


Methotrexate

Methylprednisone + cyclosporine
This is the correct answer


Mycophenolate mofetil


Tacrolimus

Acute graft versus host is the major cause of early


transplant-related morbidity and mortality. It is characterised
Score:
by
fever, rash, abnormal LFTs, diarrhoea, suppression of engraftment
and viral reactivation.
Total Answered:
Management revolves around good nutrition, weight maintenance
and immunosuppression with
methylprednisone and cyclosporin.
Feedback
Tacrolimus and mycophenolate mofetil are second line agents for
the use of acute GvHD.

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A 21-year-old lady diagnosed with severe type 1 von Willebrand
disease is planned to undergo emergency
Test Analysis
appendicectomy. 

After informing the coagulation laboratory that monitoring will


be required with vWF:RCo or vWF:Ag, which
coagulation factor is the
most appropriate for the management of this patient with von
Willebrand disease?

(Please select 1 option)



Cryoprecipitate


Desmopressin


High purity factor VIII


Intermediate purity factor VIII


Tranexamic acid

Score:
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A 21-year-old lady diagnosed with severe type 1 von Willebrand
disease i