STUDY Q&A GUIDE: FALL 2012

HEMOSTASIS DISORDERS
Items 13
A 22-year-old black woman with no previous bleeding history develops persistent bleeding of her
gums and tooth socket following dental surgery. Additional history demonstrates problems related
to heavy menses that significantly resolves when she is taking oral contraceptives and recurs to the
same level of severity when she discontinues the medication. In addition, she has a history of easy
bruisability and recurrent epistaxis. Hematologic studies reveal the following:
Partial thromboplastin time (PTT)
55 sec (2840 sec)
Prothrombin time (PT)
12 sec (1115 sec)
Factor VIII
30% (50150%)
Bleeding time
15 min (27 min)
Platelet count
300,000 mm3 (150,000400,000 mm)
Hemoglobin
13.0 g/dL (12.016.0 g/dL)
1.

Which of the following laboratory tests is most useful in confirming the cause of her bleeding
disorder?
A.
Leukocyte count
B.
Ristocetin cofactor assay
C.
Hemoglobin electrophoresis
D.
Sickle cell preparation
E.
Serum ferritin
Answer: B: ristocetin cofactor assay: the patient has VWD and this is the most sensitive test. The
prolonged PTT, decreased VIII: C and VIII:Ag, and prolonged BT also lend to that diagnosis.
Recall that if VWF is decreased, VIII is also decreased because VWF complexes with VIII in the
circulation and prevents it from degrading. Note the mixture of platelet and coagulation problems
(bleeding socket, easy bruising, epistaxis, menorrhagia).
A.
Leukocyte count: she does not have leukemia, since her Hgb and platelet count are both normal.
C.
Hemoglobin electrophoresis: there is no hemoglobinopathy
D.
Sickle cell preparation: she does not have sickle disease. She could have trait, but that would not
explain any of her current problems.
E.
Serum ferritin: it does not confirm her bleeding disorder, but it could be decreased and at an early
stage due to her history of menorrhagia
2.

Which of the following best describe the patient's bleeding disorder? SELECT 3
A.
Platelet adhesion molecule defect
B.
Coagulation defect
C.
Qualitative platelet disorder
D.
Defect in fibrinolysis
E.
Inhibitor defect
Answers: A, B, C:
A. Platelet adhesion molecule defect: decreased VIII:vWF
B. Coagulation defect: decreased VIII
C. Qualitative platelet disorder: normal platelet count but prolonged BT
D. Defect in fibrinolysis: wrong history. It is not DIC or a primary fibrinolysis
E. Inhibitor defect: wrong history

3.

If this patient did not want to remain on oral contraceptives, which of the following would be the
best treatment for her bleeding disorder?
A.
Desmopressin acetate
B.
Cryoprecipitate
C.
Factor VIII concentrate
D.
Warfarin
E.
Heparin
Answer: A: desmopressin acetate: this increases release of VWF from Weibel Palade bodies, which
combines with factor VIII in the blood to prevent its degradation just like the estrogen does in the oral
contraceptive pill
B. Cryoprecipitate: it is a blood product and has all the blood product risks (e.g., HCV, HIV, etc.; it
contains increased concentrations of fibrinogen, VIII and XIII)
C. Factor VIII concentrate: rarely necessary, since the coagulation defect is mild in VWD
D. Warfarin: anticoagulation is unnecessary
E. Heparin: as above
Items 46
A 23-year-old man requires a root canal for an abscessed tooth. On the day prior to the procedure, he
develops a severe nosebleed, which prompts his dentist to order a few laboratory studies, which are
listed below.
Partial thromboplastin time (PTT)
35 sec (2840 sec)
Prothrombin time (PT)
13 sec (1115 sec)
Bleeding time
16 min (27 min)
Platelet count
300,000 mm3 (150,000400,000 mm)
Hemoglobin
15.5 g/dL (13.517.5 g/dL)
4.

Which of the following hemostasis abnormalities is most likely present in this patient?
A.
Von Willebrand's disease
B.
Hemophilia A
C.
Factor IX deficiency
D.
Acquired platelet defect
E.
Acquired vascular defect
Answer: D. Acquired platelet defect: patient is probably on an NSAID for pain an no temporary platelet
plug is being formed. Note the prolonged BT and normal coagulation studies.
A. Von Willebrand's disease: PTT is normal
B. Hemophilia A: as above
C. Factor IX deficiency: as above
E. Acquired vascular defect: no signs of a vascular defect
5.

Which of the following best describe the mechanisms for this patient's bleeding disorder? SELECT
3
A.
Platelet adhesion molecule defect
B.
Defect in platelet aggregation
C.
Decreased synthesis of TXA2
D.
Defect in producing a temporary platelet plug
E.
Defect in converting fibrinogen into fibrin
Answers: B, C, D:
B. Defect in platelet aggregation: platelet is not synthesizing TXA 2
C. Decreased synthesis of TXA2

D. Defect in producing a temporary platelet plug: there is no temporary platelet plug

A. Platelet adhesion molecule defect: this is not VWD. The PTT is normal.
E. Defect in converting fibrinogen into fibrin: this is not a coagulation disorder
6.

If this patient experienced a life-threatening bleed related to his disorder, which of the following
treatments would stop the bleeding?
A.
Infusion of fresh frozen plasma
B.
Infusion of platelets
C.
Infusion of cryoprecipitate
D.
Infusion of packed RBCs
E.
Infusion of whole blood
Answer: B. Infusion of platelets: only platelets that are functioning can stop the bleeding
A.
Infusion of fresh frozen plasma: it is not a coagulation defect
C.
Infusion of cryoprecipitate: as above
D.
Infusion of packed RBCs: does not contain enough platelets
E.
Infusion of whole blood: as above
7.

A 53-year-old alcoholic has epigastric distress, widespread ecchymoses, and mucosal bleeding. A
rectal exam demonstrates a bloody stool with a greasy consistency. A flat plate of the abdomen
exhibits fine calcifications in the left upper quadrant. The CBC uncovers a mild macrocytic anemia.
The prothrombin time is 19 sec (1115 sec) and corrects to 14 sec after an intramuscular injection
of vitamin K. Which of the following mechanisms BEST explains the physical and laboratory
findings in this patient?
A.
Vitamin K deficiency secondary to deficiency of bile salts
B.
Vitamin K deficiency secondary to chronic pancreatitis
C.
Bile salt deficiency secondary to chronic liver disease
D.
Folate deficiency secondary to a poor diet
E.
Multiple coagulation deficiencies secondary to chronic liver disease
Answer: B. Vitamin K deficiency secondary to chronic pancreatitis: chronic pancreatitis (dystrophic
calcification of the pancreas) is causing malabsorption of the fat soluble vitamins, including vitamin K.
The PT corrects with IM vitamin K indicating that the liver is synthesizing the vitamin K-dependent
precursors and vitamin K is -carboxylating the coagulation factors.
A. Vitamin K deficiency secondary to deficiency of bile salts: the patient does not have cirrhosis to
explain bile salt deficiency
C. Bile salt deficiency secondary to chronic liver disease: the fact that the PT corrected to normal,
exonerates chronic liver disease
D. Folate deficiency secondary to a poor diet: folate deficiency explains the macrocytic anemia, but it
does not explain ecchymoses and steatorrhea
E. Multiple coagulation deficiencies secondary to chronic liver disease: there is no chronic liver disease

Items 89
A 63-year-old man with urinary retention secondary to benign prostatic hyperplasia develops fever
and chills shortly after insertion of an indwelling catheter. Physical exam demonstrates warm skin
and a bounding pulse. Within 24 hours, he begins oozing blood out of venipuncture sites and from
his mucous membranes. Ecchymoses appear over his trunk and extremities. His urine output
decreases to <400 mL/day. Laboratory studies show the following:
Hemoglobin
10 g/dL (13.517.5 g/dL)
Leukocyte count
2,000/mm3 (4,50011,000/mm3)
Platelet count
140,000/mm3 (150,000400,000/mm3)
Partial thromboplastin time
42 sec (2840 sec)
Prothrombin time
18 sec (1115 sec)
Plasma fibrinogen
150 mg/dL (200400 mg/dL)
Fibrin(ogen) degradation products
>10 g/mL (<10 g/mL)
D-dimers
positive (negative)
Blood cultures
pending
Serum blood urea nitrogen
80 mg/dL (718 mg/dL)
Serum creatinine
8 mg/dL (0.61.2 mg/dL)
8.

Which of the following apply to this case? SELECT 4

A.
Thrombotic thrombocytopenic purpura
B.
Disseminated intravascular coagulation
C.
Consumption of coagulation factors
D.
Prerenal azotemia
E.
Secondary fibrinolysis
F.
Decreased production of platelets
G.
Increased total peripheral resistance
H.
Endotoxic shock
Answers: B, C, E, H: the patient has DIC and is in renal failure (BUN/creatinine ratio < 15)
B. Disseminated intravascular coagulation: endotoxic shock is the most common cause. Note oozing from
all sites.
C. Consumption of coagulation factors: I, II, V, VIII
E. Secondary fibrinolysis: via XIIa converting prekallikrein to kallikrein, which, in turn, activates
plasminogen
H. Endotoxic shock: E. coli, is the most common organism.
A. Thrombotic thrombocytopenic purpura: the PTT and PT are not prolonged in TTP and the fibrinogen
is normal.
D. Prerenal azotemia: the ratio is < 15:1 not 15:1
F. Decreased production of platelets: they are being consumed
G. Increased total peripheral resistance: it is decreased due to vasodilatation from NO, anaphylatoxins,
PGI2.
9.

Which of the following is the most effective treatment for this patient?
A.
Fresh frozen plasma
B.
Packed red blood cells
C.
Platelet concentrates
D.
Low dose heparin
E.
Antibiotics

Answer: E. Antibiotics: in DIC, the most effective Rx is to Rx the cause of the DIC, in this case,
endotoxic shock secondary to E. coli. The blood components listed below are also indicated, but
essentially feed the fire and keep the patient alive until the underlying disease is eradicated.
A. Fresh frozen plasma: provides all the coagulation factors
B. Packed red blood cells: corrects anemia
C. Platelet concentrates: corrects thrombocytopenia
D. Low dose heparin: prevents fibrin clots from forming, hence they are not consumed anymore
10.

Which of the following laboratory test results are more often associated with classical von
Willebrand's disease rather than mild hemophilia A? SELECT 2
A.
Normal prothrombin time
B.
Decreased factor VIII activity
C.
Abnormal ristocetin cofactor assay
D.
Prolonged partial thromboplastin time
E.
Prolonged bleeding time
F.
Normal platelet count
G.
Response to desmopressin acetate
Answers: C, E:
C. Abnormal ristocetin cofactor assay: measures presence of VIII:vWF
E. Prolonged bleeding time: due to platelet adhesion defect,
A. Normal prothrombin time: both have this
B. Decreased factor VIII activity: as above
D. Prolonged partial thromboplastin time: as above
F. Normal platelet count: as above
G. Response to desmopressin acetate: as above
Items 1119
A.
B.
C.
D.
E.
F.
G.

Platelet count
Decreased
Decreased
Normal
Normal
Normal
Normal
Normal

Bleeding time
Prolonged
Prolonged
Prolonged
Normal
Normal
Normal
Prolonged

PTT
Normal
Prolonged
Prolonged
Prolonged
Prolonged
Normal
Normal

PT
Normal
Prolonged
Normal
Normal
Prolonged
Prolonged
Normal

11. A 4-yr-old child has eaten rat poison and is hemorrhaging

Answer: E rat poison is warfarin, which inhibits epoxide reductase, hence keeping vitamin K in its
inactive form. This blocks activation of all the vitamin K-dependent factors (II, VI, IX, X). Both PT and
PTT are prolonged (II, X are both in the common pathway).
Platelet count Bleeding time PTT
PT
E.
Normal
Normal
Prolonged
Prolonged
12. A 54-yr-old man is on heparin to prevent deep venous thrombosis
Answer: E heparin enhances AT III, which neutralizes most intrinsic factors and most of the final
common pathway factors, hence both PT and PTT are prolonged
E.
Normal
Normal
Prolonged
Prolonged

A.
B.
C.
D.
E.
F.
G.

Platelet count
Decreased
Decreased
Normal
Normal
Normal
Normal
Normal

Bleeding time
Prolonged
Prolonged
Prolonged
Normal
Normal
Normal
Prolonged

PTT
Normal
Prolonged
Prolonged
Prolonged
Prolonged
Normal
Normal

13. A 43-yr-old man has a history of factor VII deficiency

Answer: F
Platelet count Bleeding time PTT
F.
Normal
Normal
Normal

PT
Normal
Prolonged
Normal
Normal
Prolonged
Prolonged
Normal

PT
Prolonged

14.

A 28-yr-old man has been envenomated by a rattlesnake and is bleeding from all orifices and all
needle stick sites
Answer: B: the patient has DIC. BT is prolonged due to Decreased platelets and FDPs interfering with
aggregation.
B.
Decreased
Prolonged
Prolonged
Prolonged
15. A child has eaten raw hamburgers and now has a hemolytic anemia and renal failure
Answer: A: HUS due to 0157: H7 serotype of E. coli: platelet, not coagulation factor consumption.
Platelet count Bleeding time PTT
PT
A.
Decreased
Prolonged
Normal
Normal
16. A woman has menorrhagia, easy bruising, and epistaxis
Answer: C: patient has VWD
Platelet count Bleeding time PTT
PT
C.
Normal
Prolonged
Prolonged
Normal
17. A man has a family history of a bleeding disorder which began with his mother's father
Answer: D: hemophilia A, maternal father transmits the disease to all his daughter's (SXR trait) who are
asymptomatic carriers. The daughters transmit the gene to 50% of their sons.
Platelet count Bleeding time PTT
PT
D.
Normal
Normal
Prolonged
Normal
18. A 49-yr-old woman with fibromyalgia is taking NSAIDS for pain
Answer: G: NSAIDs block platelet cyclooxygenase
Platelet count Bleeding time PTT
PT
G.
Normal
Prolonged
Normal
Normal
19.

A 49-yr-old man, who is taking heparin, develops epistaxis and petechial lesions over his torso on
day 6 after therapy
Answer: B: the patient has type II variant of heparin induced thrombocytopenia, where heparin binds to
PF4 and an IgG antibody reacts against the complex (type II hypersensitivity)
Platelet count Bleeding time PTT
PT
B.
Decreased
Prolonged
Prolonged
Prolonged

20.

An afebrile 8-year-old boy develops epistaxis 1 week after an upper respiratory infection. Physical
examination demonstrates scattered petechia and ecchymoses over his trunk. There is no
lymphadenopathy or hepatosplenomegaly. A stool for occult blood is negative. His CBC exhibits a
hemoglobin of 13 g/dL (12.015.0 g/dL), leukocyte count of 8,500/mm3 (4,50013,500/mm3) with
a normal differential count, and a platelet count of 10,000/mm3 (150,000400,000/mm3). Which of
the following apply to this case? SELECT 3
A.
Endothelial cell damage secondary to a toxin
B.
Megakaryocytes are present in the marrow
C.
Type II hypersensitivity reaction against platelets
D.
Best treated with corticosteroids
E.
Multiple coagulation factor deficiencies
Answers: B, C, D: the patient has acute ITP due to IgG antibodies directed against platelet GPIIb/IIIa
receptors for fibrinogen
B. Megakaryocytes are present in the marrow: they are usually increased
C. Type II hypersensitivity reaction against platelets
D. Best treated with corticosteroids
A. Endothelial cell damage secondary to a toxin: that is either TTP or HUS. In TTP it is associated with
absence of VWF metalloprotease, which degrades VWF, while in HUS it is due to the Shiga toxin.
E. Multiple coagulation factor deficiencies: the PT and PTT are normal with primary platelet problems
and would likely be prolonged with coagulation factor deficiencies.
21.

A 38-year-old woman develops fever, mental status alterations, and epistaxis. Physical examination
demonstrates retinal hemorrhages, widespread petechia and ecchymoses, and a positive stool for
occult blood. There is no lymphadenopathy or hepatosplenomegaly. The CBC report indicates a
normocytic anemia and a normal leukocyte count. The corrected reticulocyte count is 12%. The
peripheral smear exhibits numerous fragmented RBCs, polychromasia, and a reduced number of
platelets. A bone marrow biopsy contains vascular channels containing platelet thrombi. There is a
positive urine dipstick for blood and numerous RBCs are present in the sediment. The serum blood
urea nitrogen is 40 mg/dL (718 mg/dL) and the serum creatinine is 4 mg/dL (0.61.2 mg/dL).
Which of the following apply to this case? SELECT 3
A.
Endothelial cell damage secondary to a toxin
B.
Type II hypersensitivity reaction
C.
Consumption of coagulation factors
D.
Consumption of platelets
E.
Intravascular hemolytic anemia
F.
Prerenal azotemia
Answers: A, D, E: the patient has TTP remember that it is not DIC. It is an acquired or genetic
deficiency in vWF-cleaving metalloprotease in endothelial cells. Increase in circulating multimers of
vWF increases platelet adhesion to areas of endothelial injury at arteriole-capillary junctions. Platelets
are consumed owing to production of platelet thrombi in areas of injury (not DIC). Enhanced by other
factors that damage endothelial cells (e.g., clopidogrel, hypertension). The PT and PTT are normal.
A. Endothelial cell damage secondary to a toxin
D. Consumption of platelets
E. Intravascular hemolytic anemia: microangiopathic with schistocytes.
B. Type II hypersensitivity reaction: no antibodies are involved
C. Consumption of coagulation factors: DIC
F. Prerenal azotemia: the ratio is 10/1 and represents acute renal failure (< 15/1)

22.

A patient is taking warfarin to prevent deep venous thrombosis. Which of the following vitamin K
dependent factors are not detected by the PT? SELECT 2
A.
II
B.
VII
C.
IX
D.
X
E.
Protein C and S
Answers C, E:
C. IX is in the intrinsic system
E. Proteins C and S are not detected by either the PT or PTT but must be separately measured
23.

Which of the following characterize BOTH classical von Willebrand's disease and mild hemophilia
A? SELECT 4
A.
Bleeding from superficial scratches
B.
Bleeding post-molar tooth extraction
C.
Palpable purpura
D.
Recurrent epistaxis
E.
Bleeding into joints
F.
Normal PT
G.
Normal platelet count
Answers: B, D, F, G: the VIII:C deficiency in VWD is always mild.
B. Bleeding post-molar tooth extraction: yes, VWD has both a platelet abnormality and coagulation
deficiency
D. Recurrent epistaxis
F. Normal PT
G. Normal platelet count
A. Bleeding from superficial scratches: hemophilia A does not have this
C. Palpable purpura: neither have this, since it represents immunocomplex vasculitis of small vessels
E. Bleeding into joints: only severe hemophilia A would have this
24.

Aspirin has its greatest benefit in the SELECT 2

A.
treatment of von Willebrand's disease
B.
treatment of disseminated intravascular coagulation
C.
prevention of an atherosclerotic stroke
D.
treatment of deep venous thrombosis
E.
prevention of deep venous thrombosis
F.
prevention of coronary artery thrombosis
Answers: C, F.
C. prevention of an atherosclerotic stroke: it prevents a platelet thrombus from developing over the plaque
F. prevention of coronary artery thrombosis: same reason as for C.
A. treatment of von Willebrand's disease: it would make it worse
B. treatment of disseminated intravascular coagulation: it would make it worse
D. treatment of deep venous thrombosis: heparin and warfarin are used for this
E. prevention of deep venous thrombosis: heparin and/or warfarin are used for this

25.

Vitamin K deficiency is an expected finding in which of the following clinical settings? SELECT 4
A.
Patient with steatorrhea secondary to gluten sensitivity
B.
Patient on intravenous heparin therapy
C.
Patient with sigmoid diverticulosis
D.
Patient on broad spectrum antibiotics
E.
Newborn who is 3 days old
F.
Alcoholic with cirrhosis and chronic pancreatitis
Answers: A, D, E, F: broad spectrum antibiotics is the MCC in a hospital setting.
A. Patient with steatorrhea secondary to gluten sensitivity: this produces malabsorption of vitamin K
D. Patient on broad spectrum antibiotics
E. Newborn who is 3 days old: all the vitamin K from mommy is gone and no bacteria have colonized the
bowel of the baby by this time to synthesize vitamin K
F. Alcoholic with cirrhosis and chronic pancreatitis: bile salt deficiency and inability to activate vitamin K
by epoxide reductase from cirrhosis and maldigestion of fats from chronic pancreatitis lead to
malabsorption of vitamin K
B. Patient on intravenous heparin therapy: no effect on K
C. Patient with sigmoid diverticulosis: no effect on K synthesis
26.

Which of the following characterizes hemostasis findings in mild hemophilia A rather than those
associated with ingestion of aspirin? SELECT 3
A.
Normal prothrombin time
B.
Normal ristocetin cofactor assay
C.
Normal platelet count
D.
Hemostasis defect corrected by infusing fresh frozen plasma
E.
Prolonged PTT
F.
Normal bleeding time
G.
Bleeding from superficial scratches
Answers: D, E, F:
D. Hemostasis defect corrected by infusing fresh frozen plasma: FFP provides factor VIII:C but not
platelets to correct the prolonged BT from aspirin
E. Prolonged PTT: it is normal with aspirin
F. Normal bleeding time: it is normal in hemophilia but prolonged with aspirin
A. Normal prothrombin time: both have this
B. Normal ristocetin cofactor assay: both have this
C. Normal platelet count: both have this
G. Bleeding from superficial scratches: only aspirin has this
27.

A 19-year-old woman has a long history of menorrhagia that is exacerbated when taking aspirin or
NSAIDs for her menstrual cramps and diminished when taking an oral contraceptive. Her serum
ferritin is decreased, PT normal, PTT prolonged, bleeding time is slightly prolonged, and the
platelet count is normal. Which of the following would you expect in this case?
A.
Normal serum TIBC
B.
Normal ristocetin cofactor assay
C.
No correction of the prolonged PTT when she is on an oral contraceptive
D.
Correction of the bleeding time when she does not take aspirin or a NSAID
E.
Correction of the PTT when adding 0.5 mL of normal plasma to 0.5 mL of her plasma in a
test tube
Answer: E: Correction of the PTT when adding 0.5 mL of normal plasma to 0.5 mL of her plasma in a
test tube: she has VWD and iron deficiency from the menorrhagia. The mixing study would correct the
PTT. Oral contraceptives helped her by increasing the synthesis of all the factor VIII molecules.
A. Normal serum TIBC: it should be increased in iron deficiency
9

B. Normal ristocetin cofactor assay: it should be abnormal due to lack of VIII:vWF

C. No correction of the prolonged PTT when she is on an oral contraceptive: it would correct, since the
synthesis of VIII:C is increased
D. Correction of the bleeding time when she does not take aspirin or a NSAID: she has VWD and a
platelet adhesion defect
28.

An 18-year-old smoking male has deep venous thrombosis (DVT) involving the right lower calf.
He has a family history of recurrent DVTs and pulmonary emboli in his mother and maternal
grandfather. His PTT and PT are normal prior to receiving a standard dose of heparin intravenously.
The PTT remains normal 1 hour after infusing heparin. The pathogenesis of his hemostasis
abnormality is most likely related to
A.
antiphospholipid antibodies
B.
a thrombohemorrhagic disorder
C.
a deficiency of antithrombin III
D.
a deficiency of a vitamin K-dependent factor
E.
antibodies directed against heparin
Answer: C. a deficiency of antithrombin III: the patient has hereditary AT III deficiency. Heparin cannot
anticoagulate without AT III. The pearl is the lack of increase in the PTT with heparin. Rx is to give
massive doses of heparin, which will activate what little ATIII is present.
A. antiphospholipid antibodies: this usually occurs in patients with SLE; PTT would prolong with heparin
B. a thrombohemorrhagic disorder: this is not DIC
D. a deficiency of a vitamin K-dependent factor: this is not protein C or S deficiency. Heparin would have
prolonged the PTT.
E. antibodies directed against heparin: that produces thrombocytopenia
29.

Which of the following cells produces a chemical that is operative in normal hemostasis in small
vessel injury, Prinzmetal's angina, and wheezing in patient's with a pulmonary embolus?
A.
Endothelial cell
B.
Macrophage
C.
Platelet
D.
Neutrophils
E.
Hepatocyte
Answer: C. Platelet: it produces TXA2 is a vasoconstrictor, bronchoconstrictor, and a platelet aggregator
A. Endothelial cell: it is operative in normal hemostasis in small vessel injury by providing VIII:vWF, but
no to the rest
B. Macrophage: it is not involved in hemostasis at all
D. Neutrophils: as above
E. Hepatocyte: it does produce coagulation factors but no, it does not have anything to do with angina or a
PE

10

30.

A 22-year-old woman with postpartum bleeding and a pre-transfusion Hgb of 6 gm/dL is still
bleeding after infusion of 2 units of packed RBCs. The PT is normal, PTT prolonged, platelet count
normal, and bleeding time is normal. The D-dimer assay is negative. A factor deficiency is
suspected, so a mixing study is performed using 0.5 mL of her plasma mixed with 0.5 mL of
normal plasma. The PTT remains prolonged. You suspect the patient has...
A.
a platelet adhesion defect
B.
a thrombohemorrhagic disorder
C.
decreased synthesis of an extrinsic system coagulation factor
D.
decreased synthesis of an intrinsic system coagulation factor
E.
an antibody directed against an intrinsic system coagulation factor
Answer: E. an antibody directed against an intrinsic system coagulation factor: the patient most likely
has a circulating anticoagulant against factor VIII. Note how the PTT did not correct with normal plasma
A. a platelet adhesion defect: it is not VWD because mixing studies would have corrected the PTT by
providing VIII
B. a thrombohemorrhagic disorder: it is not DIC
C. decreased synthesis of an extrinsic system coagulation factor: it is not factor VII deficiency
D. decreased synthesis of an intrinsic system coagulation factor: it is due to destruction of VIII not a
decreased production of VIII
31.

A 23-year-old woman has a history of recurrent epistaxis and iron deficiency with guaiac positive
stools. Other members in her family of both sexes have similar problems. Her bleeding time, PT,
PTT, and platelet count are all normal. Examination of her oral mucosa and endoscopic evaluation
of her upper GI tract demonstrates a similar abnormality. The pathogenesis of her abnormality is
most likely related to
A.
a coagulation factor deficiency
B.
a vascular disorder
C.
a quantitative platelet disorder
D.
an autoimmune disorder
E.
a platelet adhesion defect
Answer: B. a vascular disorder: she has Osler-Weber-Rendu disease alias hereditary telangiectasia, an AD
disorder. Telangiectasis in the GI tract lead to chronic iron deficiency.
A. a coagulation factor deficiency: PT and PTT are normal in OWR
C. a quantitative platelet disorder: BT is normal in OWR
D. an autoimmune disorder: OWR is a genetic disease
E. a platelet adhesion defect: it is not VWD
32.

A 58-year-old alcoholic man has fatigue, hepatomegaly with a nodular surface and a positive stool
guaiac. His prostate gland is diffusely enlarged but no areas of induration are present. Hematologic
findings show thrombocytosis, a mild microcytic anemia, and a decreased serum ferritin. A
biochemical profile exhibits a normal bilirubin, normal transaminases (AST and ALT), increased
alkaline phosphatase, increased -glutamyltransferase, and an increased LDH. Which of the
following tests is most indicated in this patient to arrive at the cause of his anemia and liver
function abnormalities?
A.
Bone marrow aspirate and biopsy
B.
CT scan of the liver
C.
Colonoscopy
D.
Hepatitis serological tests
E.
Prostate specific antigen
Answer: C. Colonoscopy: he has colon cancer metastatic to liver (note increased ALP, GGT, and LDH,
but normal total bilirubin and transaminases indicating a malignant space occupying lesion). Note the iron

11

deficiency anemia with thrombocytosis. Thrombocytosis could be due to the malignancy or the iron
deficiency.
A. Bone marrow aspirate and biopsy: not necessary
B. CT scan of the liver: it would be necessary to document the metastasis, but no, it does not document
the cause of the anemia, which is colon cancer
D. Hepatitis serological tests: the enzyme profile is wrong for hepatitis (bilirubin and transaminases
would be increased).
E. Prostate specific antigen: he probably has hyperplasia
Items 3334
A.
B.
C.
D.
E.
F.
G.
H.

Bleeding time
Prolonged
Prolonged
Prolonged
Prolonged
Normal
Prolonged
Normal
Normal

Platelet count
Normal
Normal
Normal
Decreased
Normal
Decreased
Normal
Normal

PT
Normal
Normal
Prolonged
Normal
Prolonged
Prolonged
Normal
Normal

PTT
Normal
Prolonged
Prolonged
Normal
Prolonged
Prolonged
Normal
Prolonged

33.

A 22-year-old type 1 diabetic with chronic renal disease has a hemorrhagic diathesis that is
reversible with dialysis
Answer: A: uremics have problems with platelet aggregation due to inhibition of platelet factor 3 by
organic acids related to the renal failure. Estrogen, desmopressin, and hemodialysis correct the
abnormality.
Bleeding time
Platelet count
PT
PTT
A.
Prolonged
Normal
Normal
Normal
34.

A 32-year-old woman with SLE has epistaxis, petechia, and nonpalpable purpura. Splenomegaly is
present on physical exam. Examination of her peripheral blood demonstrates an absence of
platelets. No schistocytes are noted. She has a severe normocytic anemia with an increased
corrected reticulocyte count. The direct Coomb's test on her RBCs is positive.
Answer: D: secondary cause of chronic idiopathic thrombocytopenic purpura
Bleeding time
Platelet count
PT
PTT
D.
Prolonged
Decreased
Normal
Normal
35.

A 52-year-old alcoholic with a hemorrhagic diathesis has micronodular cirrhosis and chronic
pancreatitis. His PT is prolonged and does not correct to normal after giving an intramuscular
injection of vitamin K. Which of the following best explains the lack of correction of the patients
prolonged PT after an injection of vitamin K?
A.
Chronic pancreatitis leading to malabsorption of fat soluble vitamins
B.
Bile salt deficiency from liver disease leading to malabsorption of fat soluble vitamins
C.
Antibiotic destruction of colonic bacteria leading to decreased vitamin K synthesis
D.
Decreased liver synthesis of coagulation factors leading to an anticoagulated state
E.
Decreased carboxylation of vitamin K dependent coagulation factors due to vitamin K
deficiency
Answer: D. Decreased liver synthesis of coagulation factors leading to an anticoagulated state: cirrhosis
is causing a generalized deficiency of coagulation factors, including the vitamin K-dependent factors,
hence giving vitamin K will not correct the prolonged PT

12

A. Chronic pancreatitis leading to malabsorption of fat soluble vitamins: the PT would have corrected
after giving K IM
B. Bile salt deficiency from liver disease leading to malabsorption of fat soluble vitamins: as above
C. Antibiotic destruction of colonic bacteria leading to decreased vitamin K synthesis: as above
E. Decreased carboxylation of vitamin K dependent coagulation factors due to vitamin K deficiency: as
above
36.

The pathogenesis of hemorrhagic skin necrosis associated with warfarin therapy is most closely
associated with
A.
antibodies directed against warfarin
B.
a drug hypersensitivity reaction
C.
immune vasculitis secondary to warfarin
D.
protein C deficiency in the patient
E.
antithrombin III deficiency in the patient
Answer: D. protein C deficiency (also protein S) in the patient: heterozygote carriers for protein C
become homozygote when given warfarin in ~68 hrs owing to the short half-life of previously carboxylated protein C. The patient is now hypercoagulable, since V and VIII are increased and
fibrinolysis is decreased.
A. antibodies directed against warfarin: doesn't exist
B. a drug hypersensitivity reaction: not the most common cause
C. immune vasculitis secondary to warfarin: doesn't exist
E. antithrombin III deficiency in the patient: produces deep vein thrombosis
37.

Which of the following gastrointestinal disorders is commonly associated with a prolonged PT?
SELECT 4
A.
Bacterial overgrowth in the duodenum
B.
Crohns disease involving the terminal ileum
C.
Chronic pancreatitis with maldigestion of fat
D.
Osmotic diarrhea secondary to laxative abuse
E.
Celiac disease involving the jejunum and terminal ileum
Answers: A, B, C, E: all of these have malabsorption of fat soluble vitamins except for laxative abuse
A. Bacterial overgrowth in the duodenum: depletes bile salts
B. Crohns disease involving the terminal ileum: depletes bile salts
C. Chronic pancreatitis with maldigestion of fat: malabsorption of vitamin K
E. Celiac disease involving the jejunum and terminal ileum: malabsorption of vitamin K
D. Osmotic diarrhea secondary to laxative abuse: does not cause vitamin K malabsorption
38.

Which of the following represents a small vessel vasculitis rather than a platelet or coagulation
abnormality?
A.
Bleeding from superficial scratches
B.
Intramuscular hematoma
C.
Easy bruiseability
D.
Palpable purpura
E.
Hemarthrosis
Answer: D. Palpable purpura: the majority are due to immunocomplexes (type III HR)
A. Bleeding from superficial scratches: platelet abnormality
B. Intramuscular hematoma: coagulation factor abnormality
C. Easy bruiseability: platelet/coagulation factor abnormality
E. Hemarthrosis: coagulation factor abnormality

13

39.

The rationale for beginning heparin and warfarin at the same time is that...
A.
previously activated vitamin K-dependent factors have long half-lives
B.
inactivation of epoxide reductase by warfarin requires 35 days
C.
warfarin enhances activation of antithrombin III by heparin
D.
warfarin reduces the complications associated with heparin
E.
heparin requires 24 hrs to fully anticoagulate the patient
Answer: A. previously activated vitamin K-dependent factors have long half-lives: the shortest are factor
VII and protein C and the longest is prothrombin
B. inactivation of epoxide reductase by warfarin requires 35 days: it is immediate
C. warfarin enhances activation of antithrombin III by heparin: no
D. warfarin reduces the complications associated with heparin: no
E. heparin requires 24 hrs to fully anticoagulate the patient: it is immediate
40.

A 75-year-old woman in a nursing home has non-palpable ecchymoses limited to the back of her
hands. A CBC is unremarkable. The patient most likely has
A. Henoch-Schoenlein's purpura
B.
autoimmune thrombocytopenia
C.
senile purpura
D. scurvy
E.
been abused
Answer: C. senile purpura: age-dependent, meaning all of us have this to look forward to
A. Henoch-Schoenlein's purpura: this produces palpable purpura due to a vasculitis
B. autoimmune thrombocytopenia: ecchymoses would locate in other areas of the body as well
D. scurvy: as above + bleeding gums
E. been abused: ecchymoses would have to be in areas not normally traumatized
41.

A 23-yr-old woman has a positive RPR and negative FTA-ABS for syphilis. She has a history of
repeated spontaneous abortions. Which of the following apply to this patient? SELECT 4
A. Antibodies against phospholipids are present
B.
Thrombi of placental vessels caused the abortions
C.
Lupus anticoagulant caused false positive RPR
D. Stroke and deep vein thrombosis are common
E.
Anticardiolipin antibodies cross the placenta
Answers: A, B, D, E: the patient has the antiphospholipid syndrome due to the LA and anti cardiolipin
antibodies, the latter responsible for the FP RPR.
A. Antibodies against phospholipids are present
B. Thrombosis of placental vessels caused the abortions
D. Stroke and deep vein thrombosis are common
E. Anticardiolipin antibodies cross the placenta
C. Lupus anticoagulant caused false positive RPR: anticardiolipin antibodies crossreact with beef
cardiolipin used in the test system
42.

A febrile 23-year-old man with acute progranulocytic leukemia (M3) has oozing of blood from
every venipuncture site, extensive ecchymoses, and mucosal bleeding. Schistocytes are noted in the
peripheral blood. Which of the following apply to this case? SELECT 3
A.
Consumption of coagulation factors
B.
Thrombohemorrhagic disorder
C.
Negative D-dimer assay

14

D.
Negative FDP assay
E.
Thrombocytopenia
Answers: A, B, E: this leukemia is always associated with DIC. It can be successfully treated with
retinoic acid and has a t(15;17) translocation.
A. Consumption of coagulation factors
B. Thrombohemorrhagic disorder: yes, combination of multiple vessel thromboses + consumption of
clotting factors leading to an anticoagulated state
E. Thrombocytopenia
C. Negative D-dimer assay: it would be positive, because fibrin clots are present in DIC
D. Negative FDP assay: as above
43.

Which of the following is the most common type of hereditary thrombosis disorder?
A.
Antithrombin III deficiency
B.
Factor V Leiden
C.
Protein C deficiency
D.
Protein S deficiency
Answer: B. Factor V Leiden: factor V Leiden cannot be degraded by protein C and S. The other types are
less common.
44.

Which of the following normally have a procoagulant role in small vessel hemostasis? SELECT 3
A.
Protein C and S
B.
Antithrombin III
C.
Thromboxane A2
D.
Tissue thromboplastin
E.
Von Willebrand's factor
Answers: C, D, E: procoagulant means that it favors clotting in small vessels
C. Thromboxane A2: platelet aggregator and vasoconstrictor
D. Tissue thromboplastin: activates extrinsic system
E. Von Willebrand's factor: platelet adhesion factor and prevents VIII:C from degrading when it forms a
complex with VIII:C
A. Protein C and S: anticoagulant by degrading V and VIII and enhancing fibrinolysis
B. Antithrombin III: anticoagulant by neutralizing serine proteases
45.

In a patient with epistaxis, petechia, ecchymoses, and bleeding from superficial scratches and
venipuncture sites, which of the following disorders should be in your differential diagnosis?
SELECT 3
A.
Mild hemophilia A
B.
Vitamin K deficiency
C.
Von Willebrands disease
D.
Idiopathic thrombocytopenic purpura
E.
Hemolytic uremic syndrome
Answers: C, D, E: petechia and bleeding from superficial scratches is a platelet dysfunction. Epistaxis
and ecchymoses can occur in both platelet/coagulation factor disorders
C. Von Willebrands disease
D. Idiopathic thrombocytopenic purpura
E. Hemolytic uremic syndrome
A. Mild hemophilia A: coagulopathy. Would not have petechia or bleeding from scratches
B. Vitamin K deficiency: coagulopathy. Would not have petechia or bleeding from scratches.

15

46.

Which of the following characterize heparin rather than warfarin?

A.
Autoimmune thrombocytopenia
B.
Bleeding from the GI and GU tract
C.
Prolonged PTT
D.
Prevents venous clot formation
E.
Delayed anticoagulation
F.
Overanticoagulation best reversed with fresh frozen plasma
Answer: A. Autoimmune thrombocytopenia: type II variant
B. Bleeding from the GI and GU tract: both
C. Prolonged PTT: both
D. Prevents venous clot formation: both
E. Delayed anticoagulation: only warfarin
F. Over-anticoagulation best reversed with fresh frozen plasma: only warfarin
47.

Which of the following characterize warfarin rather than heparin? SELECT 3

A.
Full anticoagulation is delayed
B.
Prolongs the PT
C.
Synthesized by colonic bacteria
D.
Blocks epoxide reductase
E.
Over-anticoagulation best reversed with protamine sulfate
Answers A, C, D:
A. Full anticoagulation is delayed: waiting for previously -carboxylated prothrombin to disappear in 3-4
days
C. Synthesized by colonic bacteria
D. Blocks epoxide reductase
B. Prolongs the PT: both
E. Over-anticoagulation best reversed with protamine sulfate: only heparin
48.

In primary fibrinolysis you would expectSELECT 2

A.
thrombocytopenia
B.
prolonged PT and PTT
C.
normal bleeding time
D.
presence of D-dimers
E.
presence of FDPs
Answers: B, E:
B. prolonged PT and PTT: plasmin degrades coagulation factors
E. presence of FDPs: plasmin cleaves fibrinogen into degradation products
A. thrombocytopenia: normal platelet count
C. normal bleeding time: prolonged due to FDPs interfering with platelet aggregation
D. presence of D-dimers: absent since no fibrin clots with cross links are present. D-dimers would be
present if secondary fibrinolysis was present as in DIC.
49.

A 24-year-old woman with chronic idiopathic thrombocytopenia delivers a newborn baby girl, who
develops petechial lesions and ecchymoses 24 hours after delivery. What is the mechanism for the
newborn's disorder?
A.
Transplacental passage of IgG antibodies against Pl A1 positive platelets: this is not an
alloimmune thrombocytopenia
B.
Transplacental passage of IgM antibodies against Pl A1 positive platelets: IgM antibodies do
not cross the placenta.
16

C.
Transplacental passage of IgG antibodies against platelet fibrinogen receptors
D.
Transplacental passage of IgM antibodies against platelet fibrinogen receptors
Answer: C: Transplacental passage of IgG antibodies against platelet fibrinogen receptors. Newborn
infants of mothers with ITP may have transient thrombocytopenia due to transplacental passage of IgG
antibodies, which are directed against the GpIIb:IIIa fibrinogen receptors.
A. Transplacental passage of IgG antibodies against Pl A1 positive platelets: this is not an alloimmune
thrombocytopenia
B. Transplacental passage of IgM antibodies against Pl A1 positive platelets: IgM antibodies do not cross
the placenta.
D. Transplacental passage of IgM antibodies against platelet fibrinogen receptors: IgM antibodies do not
cross the placenta.
A 25-year-old woman, who is in the third trimester of her first pregnancy, is Pl A1 negative. Her
platelet count is normal. Shortly after birth, her newborn son develops petechia and ecchymoses
and a stool that is positive for occult blood. What is the mechanism for the newborn's disorder
(Discussed in Lab Medicine book page 251)?
A.
Transplacental passage of IgG antibodies against Pl A1 positive platelets: this is not an
alloimmune thrombocytopenia
B.
Transplacental passage of IgM antibodies against Pl A1 positive platelets: IgM antibodies do
not cross the placenta.
C.
Transplacental passage of IgG antibodies against platelet fibrinogen receptors
D.
Transplacental passage of IgM antibodies against platelet fibrinogen receptors
Answer: A: Transplacental passage of IgG antibodies against Pl A1 positive platelets. The patient has
neonatal alloimmune thrombocytopenic purpura. The mother is negative for Pl A1, while the newborn is
positive for the antigen. It accounts for 20% of cases of thrombocytopenia in neonates. Pl A1 is absent from
2% of the population. PlA1 negative mother develops IgG antibodies during pregnancy or from a previous
pregnancy or transfusion. In contradistinction to Rh hemolytic disease of newborn, where the firstborn
child is unaffected, in NAIT, the firstborn child is affected. Transplacental passage of IgG antibodies bind
to fetal PlA1 positive platelets leading to macrophage destruction of platelets (type II hypersensitivity).
May produce petechial hemorrhages in first few days of life or CNS hemorrhages in severe cases.
B. Transplacental passage of IgM antibodies against Pl A1 positive platelets: IgM antibodies do not cross
the placenta.
C. Transplacental passage of IgG antibodies against platelet fibrinogen receptors: the mother does not
have chronic ITP.
D. Transplacental passage of IgM antibodies against platelet fibrinogen receptors: IgM antibodies do not
cross the placenta.
50.

51.

A 35-year-old woman, who has 5 children, receives a unit of packed RBCs for a ruptured spleen
she sustained in a car accident. One week later she develops epistaxis, petechia, ecchymoses, and
bloody stools. Her platelet count is 10,000/mm3. D-dimers are absent. What is the mechanism for
her platelet disorder (Discussed in Lab Medicine book page 251)?
A.
Consumption of platelets
B.
Immune destruction of platelets
C.
Sequestration of platelets
D.
Decreased production of platelets
E.
Nonimmune destruction of platelets
Answer: B. Immune destruction of platelets. The patient has post-transfusion purpura, which primarily
occurs in multiparous women. The patient is most likely Pl A1 negative and has antibodies against PlA1 or
other platelet antigens that are present on donor platelets. Severe thrombocytopenia develops with
destruction of donor and patient platelets (unknown mechanism) 7-10 days after a blood transfusion.
A. Consumption of platelets: this occurs in TTP, HUS, and DIC
17

C. Sequestration of platelets: this occurs in patients with portal hypertension and splenomegaly.
D. Decreased production of platelets: this occurs with aplastic anemia, leukemia, or with certain types of
drugs
E. Nonimmune destruction of platelets: TTP, HUS, and DIC are examples of nonimmune destruction of
platelets.

18