PREFACE

Idiopathic thrombocytopenic purpura (ITP) is the condition of having a

low platelet count (thrombocytopenia) of no known cause (idiopathic). As most
causes appear to be related to antibodies against platelets, it is also known as
immune thrombocytopenic purpura. Although most cases are asymptomatic, very
low platelet counts can lead to a bleeding diathesis and purpura. ITP is a disorder
that affects the overall number of blood platelets rather than their function. The
normal platelet level in adults is between 150,000 and 450,000/mm3. Platelet
counts below 50,000 mm3 increase the risk of dangerous bleeding from trauma;
counts below 20,000/mm3 increase the risk of spontaneous bleeding. ITP may be
either acute or chronic. The incidence of ITP is 50-100 new cases per million per
year, with children accounting for half of that amount and the median age of
adults at the diagnosis is 56-60. In adults, ITP is considered an autoimmune
disorder, which means that the body produces antibodies that damage some of its
own products-in this case, blood platelets. In acute ITP cases patients usually
suffer from bruising; petechiae, nosebleeds and bleeding gums may occur if the
platelet count is below 20,000, compared with a normal range of 150,000-
400,000/mm3. In extreme cases, patients with ITP may bleed into the lungs, brain,
or other vital organs, leading to subarachnoid, intracerebral hemorrhage or other
internal bleeding are very serious possible complications of this disease but these
complications are unlikely in patients with the platelets count above 20,000. In
many cases, the pathogenesis is not actually idiopathic, but autoimmune, with
antibodies against platelets being detected in approximately 60% of patients. Most
often these antibodies are against platelet membrane glycoproteins IIb-IIIa or Ib-
IX, and are of the immunoglobulin G (IgG) type. The coating of platelets with
IgG renders them susceptible to opsonization and phagocytosis by splenic
macrophages.
Recent evidence suggests that the stimulus for autoantibody production in
ITP is due to abnormal T helper cells reacting with platelet antigens on the surface
of antigen presenting cells. This important finding suggests that therapies directed
toward T cells may be effective in treating ITP. The diagnosis of ITP is a diagnosis
of exclusion. First, one has to make sure that there are no other blood
abnormalities except for low platelet count and no physical signs except for signs
of bleeding. Then, the secondary causes (usually 5-10% of suspected ITP cases)
should be excluded. Secondary causes could be leukemia, medications (e.g.,
quinine, heparin), lupus erythematosus, and cirrhosis, HIV, hepatitis C, congenital
causes, antiphospholipid syndrome, von Willebrand factor deficiency and others.
In approximately 1% of cases autoimmune hemolytic anemia and immune
thrombocytic purpura coexist, which is a condition called Evans syndrome.
Complete blood count usually have platelet counts below 20,000/mm 3. The size
and appearance of the platelets may be abnormal. The red blood cell count and
white blood cell count are usually normal, although about 10% of patients with
ITP are also anemic. Bleeding time is usually prolonged. Bleeding time is
prolonged in ITP patients; however, the use of bleeding time in diagnosis is

1
discouraged by the American Society of Hematology practice guidelines as
useless.
A bone marrow examination may be performed on patients over the age of
60 and people who do not respond to treatment, or when the diagnosis is in doubt.
On examination of the bone marrow, an increase in the production of
megakaryotes is seen and can help in determining ITP. Detection of antiplatelet
antibodies in the blood is considered to confirm the diagnosis of ITP. Treatment
should be restricted to those patients with moderate or severe thrombocytopenia
who are bleeding or at risk of bleeding. It should be limited in duration unless
demonstrated that symptomatic thrombocytopenia persists. Patients with mild,
asymptomatic thrombocytopenia, discovered incidentally on a routine blood
count, should not be treated.

2
 CHAPTER I

CASE ILLUSTRATION

A. IDENTITY

a. Patient

 Name : Child RJ

 Date of birth : 09th July 2018

 Sex : Male

 Address  : Jati Jajar RT 06/03 TAPOS, Depok

 Tribe : Palembangnese 

 Religion : Islam

 Education : ­

b. Parents

Father

 Name : Mr. MI

 Date of birth : 29th April 1984

 Sex : Male

 Address  : Jati Jajar RT 06/03 TAPOS, Depok

 Tribe : Palembangnese

 Religion : Islam

 Education : High School

3
  Occupation : Employee

Mother 

 Name : Mrs. R

 Date of birth : 14th March 1987

 Sex : Female

 Address  : Jati Jajar RT 06/03 TAPOS, Depok

 Tribe : Palembangnese

 Religion : Islam

 Education : High School 

 Occupation : Housewife

B. HISTORY

History   was   taken   from   the   patient   (autoanamnesis)   at   Anggrek   II

Bhayangkara Hospital R. Said Sukanto, East­Jakarta on 17th September 2018. 

a. Chief Complaint
Reddish spots on the head from 4 days before entering the hospital .
b. Additional Complaint
Redness of the extremities and hematoma in the gums and ears .
c. History of Present Illness
Since 4 days  before entering the hospital, the patient complained of
reddish   patches   on   the   head. Then   there   is   hematoma   in   the   inferior
gingivae and the sinistral auricle. 1 day later, the patient was taken to the
Sentra Medika Hospital and then referred to the POLRI Hospital with a
diagnosis   of   suspected   ITP. At   present   the   patient   has   no   additional
complaints.

a. History of Past Illness

Disease  Age 
Upper   Respiratory   Infection   (e.g. ­

Pharyngitis/Tonsilitis)
Diarrhea  ­
Otitis  ­

4
Pneumonia  ­
Tuberculosis  ­
Seizure  ­
Heart  ­ 
Blood  ­
Diphtheria  ­
Measles  ­
Mumps  ­
Dengue fever ­
Typhoid fever  ­
Worms infection ­
Allergy  ­
Accident  ­

b. Allergy history

 Food allergy: denied

 Drugs allergy: denied

 Asthma bronchial: denied

c. Dietary history

Age Breast/formula Fruit/ Milk Steam Rice & Side

(years) milk biscuit porridge Rice dishes

0­2 ASI ad libitum ­ ­ ­ ­

months

d. Growth and Development history

First dentition : None

Gross motor

Head up : 1 month old

Prone : None

Sit : None

5
 Crawl : None

Stand : None

Walk : None

Run : None

Fine motor

Reaches for objects : None

Puts objects in mouth : None

Throw objects : None

Scribbling paper : None

Social skills

Smile : 1 month old

Hugs and kisses : None

Initiates play : None

Imitates adult activities : None

Language skills

Vocalized : 1 months old

Laughs : 2 months old

Jabbers : None

Speaks : None

Development disorder : None

Mental/emotion : Stable

e. Marital History

Mother’s Pregnancy History

6
 The mother routinely checked her pregnancy to midwife. She denied any

problem noted during her pregnancy. She took vitamins routinely given.
Child’s Birth History
Maternity care : Midwife

Mode of delivery : Normal, spontaneous, no complication

Gestational age : 9 months

Child status :

 Weight of birth  : 3100 gr

 Length of birth : 47 cm

 Head circumference : 37 cm

 Congenital anomaly  : ­
According to the mother, the baby started to cry and the baby's skin is red.

f. Immunization history

Immunizatio Frequenc Time

n y
BCG 1 time 1   month

old
Hepatitis B 1 times 0 month
DPT ­ ­
Polio ­ ­
Hib ­ ­
Measles ­ ­
Varicella ­  ­

g. Family history

 Reproduction pattern

No Age  Sex  Alive  Stillbirt Abor Death Health

h  tion  (cause) status

1 3 Male V ­ ­ ­ Patient

7
 months
 There   are   not   any   significant   illnesses   or   chronic   illnesses   in   the   family
declared.

h. History of Disease in Other Family Members / Around the House

There   is   no   one  living   around   their   home   known  for   having   the   same

condition as the patient.

C. PHYSICAL EXAMINATION

Physical examination was held on September 18th  2018 at Anggrek ward

Bhayangkara Hospital, R. Said Sukanto Jakarta

a. General Examination

General condition : Mild ill

Awareness : Composmentis

Vital sign :

 Heart rate : 140 bpm

 Respiratory rate : 26 times/min

 Temperature  : 36,5C

Anthropometry  :

 Body weight : 6,6 kg

 Body height : 64 cm 

b. Nutritional Status 

Nutritional status measured based on National Center for Health Statistics

(2000):

 WFA : 6,6/6 x100 = 110% (Good)

 HFA : 64/61 x 100 = 104% (Good)

 WFH : 6,6/7 x 100 = 85% (Mild)

8
 

9
 Conclusion: Nutrition status of the patient is good

c. Head to Toe Examination

Head :

 Measurement : Normocephalic

 Hair and scalp : Red spots on the head

 Eyes :   Pale   conjunctiva   ­/­,   icteric   sclera   ­/­,   pupil

isochor, reddish spot on sclera 

 Ears :   Normotia,   secret/cerumen   ­/­,   hyperemia   ­/­,

haematoma on auricula sinistra

 Nose : Septum deviation (­), nostril breathing (­), edema

conca ­/­, secret (­)

 Mouth

­ Lips  : Moist

­ Teeth  : Caries dentist (­)

­ Tongue : Clean, tremor (­)

­ Tonsil  : T1/T1, detritus (­), wide crypt (­) 

­ Pharynx : Hyperemia (­)

10
Neck  : No enlargement of lymph node

Thorax :

 Chest wall : Retraction (­)

 Pulmo  :

- Inspection  : Symmetric when static and dynamic 

- Palpation  : Vocal fremitus +/+

- Percussion  : Sonor +/+

- Auscultation : Vesicular +/+, rhonchi ­/­, wheezing ­/­

 Cor  :

- Inspection  : Ictus cordis can’t be seen

- Palpation  : Ictus cordis felt in ICS V Linea MCS without thrill

- Percussion :

Margin of right heart : ICS IV Linea PSD

Margin of left heart : ICS V Linea MCS

Margin of waist heart : ICS III Linea PSS

- Auscultation : S1S2 regular, murmur (­), gallop (­)

 Abdomen: 

- Inspection : Even, there is no a widening of the veins, no spider

nevi

- Auscultation : Bowel sound (+) normal 

- Palpation :   Hepar   and   lien   not   palpable,   epigastric   and

umbilical tenderness (+)

- Percussion : Tympani

- Other : Ballotement (­)

 Anal and rectum : anal exist, diaper rash (­), no abnormalities

 Genital: 

Pubic hair : ­

11
 Mons pubis : ­

 Extremities : Warm +/+/+/+, edema ­/­/­/­, CRT < 2s, normal

ROM 

 Skin : Cyanosis (­), icteric (­), petechiae(+)

 Vertebrae :   Deformity   (­),  gibbus   (­),  kyphosis  (­),  scoliosis

(­), lordosis (­)

d. Neurologic examination

 Physiologic reflex:

- Brachioradialis  : +2/+2

- Biceps  : +2/+2

- Triceps  : +2/+2

- Patella  : +2/+2

- Achilles  : +2/+2

 Pathologic reflex : ­

 Motoric  : 5 5 5 5  5 5 5 5 

    5 5 5 5  5 5 5 5

 Meningeal sign  : ­

12
Fig. Patient’s condition in hospital

13
D. LABORATORIUM EXAMINATION

a. Routine blood count (17/09/2018) at Sentra Medika Hospital (16.56)

Results  Normal

range
Haemoglobin  10,9 g/dl 14­18 g/dl
Leucocyte  9.200 u/l 5.000­10.000

u/l
Haematocrit  28% 42­52 %
Thrombocyte  15.000 /ul 150.000­

400.000 /ul
Bleeding Time 2’45” minutes 1­3 minutes
Clotting Time 9’25” minutes 6­10 minutes

b. Laboratory findings (17 September 2018) at RS POLRI

Result Normal Range
Haemoglobin 10,1 g/dl 13­16 g/dl
Leucocyte 9.100 u/l 5.000­10.000 u/l
Haematocrit 29% 40­48%
Thrombocyte 8.000/ul 150.000­

400.000/ul

c. Biochemical profile and electrolyte (18/09/2018)

Results  Normal range

14
 Albumin 4,0 g/dl 3,5­5,2 g/dl
Blood sugar 86 mg/dl <200 mg/dl

Natrium 140mmol/l 135­145 mmol/l

Potassium 4,1 mmol/l 3,5­5,0 mmol/l
Chloride  103 mmol/l 98­108 mmol/l

d. Urine (19/09/2018)

Results  Normal range 
Color Yellow 
Clarity  Clear
Reaction/ Ph 8,0 5­8,5
Density  1.010 1.000­1.030
Protein  ­ Negative 
Bilirubin  ­ Negative 
Glucose  ­ Negative 
Keton  ­ Negative
Blood/ Hb ­ Negative 
Nitrit  ­ Negative
Urobilinogen  0,1 0,1­1,0 IU
Leucocyte  ­ Negative 
Sediment:
 Leucocyte  0­2 0­5
 Erythrocyte  0­1 1­3
 Epithelia  +
 Cylinder  ­
 Crystal  ­
Other  ­

f. LABORATORIUM EXAMINATION (20­09­2018)
  
Result Normal Range
Haemoglobin 7,8 g/dl 13­16 g/dl
Leucocyte 8.200 u/l 5.000­10.000u/l

15
 Haematocrit  24% 40­48%
Thrombocyte  329.000/ul 150.000­

400.000/ul

E. SUMMARY

A baby boy aged 2 months was delivered by his parents to the R. Said

Sukanto Hospital Emergency Room with complaints of red spots on the head

since 4 days ago. In addition to red spots on the head, there is a hematoma in

the left auricula and in the inferior gingiva. From a physical examination there

were no abnormalities. Laboratory tests found platelets of 15,000 / ul patients

and decreased to 8,000 / ul.

F. WORKING DIAGNOSIS

1. Idiopathic Thrombocytopenic Purpura

2. Normal Growth Status

3. Good Nutritional Status

G. PROGNOSIS 

Quo ad vitam: dubia ad bonam

Quo ad functionam: dubia ad malam

Quo ad sanationam: dubia ad malam

H. TREATMENT 

 IVFD RL 800 cc/24 hr 

 Transfusi Tc 4unit/day (every 3 days)

 Dexamethasone 3x1 mg IV

16
FOLLOW UP

Day­2 (18­09­2018)

S               : Red   spots   on   the   face   (+),   gum   (+)   and   feet   (+). Nosebleed   (­),
bleeding gums (­)
O               :
 General appearance: look well
 Vital sign:
­  HR: 140 bpm
­  RR: 26 x / min
­  T : 36, 5  C
 Eye: pale  conjunctiva   +   /  + ,  icteric  sclerae  ­  /  ­,  sunken  ­  /   ­ ,
pupil isokor 3/3 mm, direct and indirect light response + / + and + / + ,
spotting on sclera (+)
 Mouth: coated tongue (­), dry mucousa (­)
 ENT: hyperaemic pharynx (­), T1­T1 tonsil
 Lungs: vesicular + / +, rhonki ­ / ­, wheezing ­ / ­
 Heart: S1S2 regular, murmur (­), gallop (­)
 Abdomen:
­  Inspection: even
­  Auscultation: normal bowel sound
­  Palpation:   liver   and   lien   not   palpable,   epigastric and
umbilical tenderness (­ )
­  Percussion: tympani
­  Ballotement ­ / ­
 Extremity: warm, CRT <2 s, edema (­)

17
  Skin: Reddish spots on the head, legs and hematoma on the gums
and ears (+)
A               : Idiopathic Thrombocytopeni Purpura
P               :
 IVFD RL 800 cc / 24 hr
 Transfusion of Tc 4unit / day (for 3 days)
 Dexamethason 3x1mg

Day­3 (19­09­18)

            S               : Bleeding (­), red spots on the face and head (+)
O               :
 General appearance: look well
 Vital sign:
­  HR: 107 bpm
­  RR: 26 x / min
­  T: 36.8  C
 Eye:   pale   conjunctiva + / + ,   icteric   sclerae   ­   /   ­,   sunken   ­   /   ­ ,
pupil isokor 3/3 mm, direct and indirect light response + / + and + / +
 Mouth : coated tongue (­), dry mucousa (­)
 ENT: hyperaemic pharynx (­), T1­T1 tonsil
 Lungs: vesicular + / +, rhonki ­ / ­, wheezing ­ / ­
 Heart: S1S2 regular, murmur (­), gallop (­)
 Abdomen:
­  Inspection: even
­  Auscultation: Normal bowel sound

18
 ­ 
Palpation: liver and spleen not palpable, epigastric and umbilical
tenderness (­ )
­  Percussion: tympani
­  Ballotement ­ / ­
 Extremity: warm, CRT <2 s, edema (­)
 Skin: Red spots on the head and face (+)
A               : Idiopathic Thrombocytopeni Purpura
P               :
 RL IVFD 500 cc / 24 days
 Tc Transfusion 4 units / day

Day­4 (20­08­18)

S               : Red spots on the head, feet and face (+), bleeding (­),
O               :
 General appearance: look well
 Vital sign:
­  H R: 115 bpm
­  RR: 27 x / min
­  T: 36  C
 Eye:   pale   conjunctiva   ­   /   ­,   icteric   sclerae   ­   /   ­,   sunken   ­   /   ­ ,
pupil isokor 3/3 mm, direct and indirect light response + / + and + / +

19
  Mouth : coated tongue (­), dry mucousa (­)
 ENT: hyperaemic pharynx (­), T1­T1 tonsil
 Lungs: vesicular + / +, rhonki ­ / ­, wheezing ­ / ­
 Heart: S1S2 regular, murmur (­), gallop (­)
 Abdomen:
o Inspection: even
o Auscultation: Normal bowel sound
o Palpation:   liver   and   spleen   not   palpable,   epigastric and
umbilical tenderness (­)
o Percussion: tympani
o Ballotement ­ / ­
 Extremity: warm, CRT <2 s, edema (­)
 Skin: Red spots on the chest and legs start to disappear (+)
A               : Idiopathic Thromocytopeni Purpura
P               :
 Aff infusion

20
 CHAPTER II

LITERATURE REVIEW

2. Idiopathic Thrombocytopeni Purpura

2.1 Definition 1

Immune thrombocytopenic purpura (ITP) is a clinical syndrome in which a

decreased   number   of   circulating   platelets   (thrombocytopenia)   manifests   as   a

bleeding   tendency,   easy   bruising   (purpura),   or   extravasation   of   blood   from

capillaries into skin and mucous membranes (petechiae). Although most cases of

acute   ITP,   particularly   in   children,   are   mild   and   self­limited,   intracranial

hemorrhage may occur when the platelet count drops below 10 × 109/L (< 10 ×

103/µL); [1] this occurs in 0.5­1% of children, and half of these cases are fatal.

In persons with ITP, platelets are coated with autoantibodies to platelet

membrane   antigens,   resulting   in   splenic   sequestration   and   phagocytosis   by

mononuclear macrophages. The resulting shortened life span of platelets in the

circulation,   together   with   incomplete   compensation   by   increased   platelet

production by bone marrow megakaryocytes, results in a decreased platelet count.

2.2 Epidemiology

The incidence of ITP in children is between 4.0­5.3 per 100,000, acute ITP
generally occurs in children aged 2­6 years. 7­28% of children with acute ITP
develop into chronic. Idiopathic Platelet Purpura in children develops into chronic
forms of ITP in some cases resembling typical adult ITP. Chronic ITP incidence
in children is estimated at 0.46 per 100,000 children per year.

              Chronic   incidence   of   chronic   ITP   is   58­66   new   cases   per   million

population per year (5.8 to 6.6 per 100,000) in the United States and similar found
in the UK. Idiopathic Thrombocytopenic Chronic purpura is commonly found in

21
median adults aged 40­45 years. The ratio between women and men is 1: 1 in
patients with acute ITP while in chronic ITP is 2­3: 1.

The actual number of ITP incidents is unknown, because individuals with
minor   illnesses   may   be   asymptomatic   so   they   are   undiagnosed. In   the   United
States, symptom disease occurs in around 70 adults / 1,000,000 and 50 children /
1,000,000. Patients   with   refractory   ITP   are   defined   as   an   ITP   that   fails   to   be
treated with standard dose corticosteroids and splenectomy which subsequently is
treated because the platelet count is below normal or there is bleeding. Patients
with refractory ITP were found in approximately 25­30 percent of the number of
people   with   ITP.This   group   has   an   ugly   response   to   therapy   with   significant
morbidity and mortality of approximately 16%.

 
2.3 Etiology

The cause of ITP is an autoimmune disorder so that platelet destruction in the

reticuloendothelial retic system increases. This   disorder   usually   accompanies   a

viral   infection   or   immunization   caused   by inappropriate immune   system

responses . Recently ITP is also often referred to as immune thrombocytopenic

purpura ( immune thrombocytopenic purpura ). 2

In children, most cases of immune thrombocytopenic purpura (ITP) are

acute, manifesting a few weeks after a viral illness. In adults, most cases of ITP

are   chronic,   manifesting   with   an   insidious   onset,   and   occur   in   middle­aged

women. These clinical presentations suggest that the triggering events may be

different. However, in both children and adults, the cause of thrombocytopenia

(destruction of antibody­coated platelets by mononuclear macrophages) appears to

be similar.1

1
Autoantibody stimulation

22
 In chronic ITP, for unknown reasons, membrane glycoproteins (GPs) on

the   surface   of   platelets   become   immunogenic,   stimulating   the   production   of

platelet autoantibodies. In acute ITP, the stimulus for autoantibody production is

also   unknown;   platelet   membrane   cryptantigens   may   become   exposed   by   the

stress of infection, or pseudoantigens may be formed by the passive adsorption of

pathogens on platelet surfaces.

1
Autoantibody specificity

In   persons   with   chronic   ITP,   approximately   75%   of  autoantibodies   are

directed against platelet GPIIb/IIIa or GPIb/IX GP complexes. Presumably, the

remaining 25% are directed against other membrane epitopes, including GPV,

GPIa/IIa, or GPIV.

2.4 Pathophysiology

ITP is caused by specific platelet autoantibodies binding to the platelets.

The IgG autoantibody­coated platelets undergo accelerated clearing in the spleen

and  liver after binding  Fc receptors  expressed on tissue macrophages. Platelet

destruction   is   presumably   triggered   by   antibody,   leading   to   the   formation   of

neoantigens,   thus   resulting   in   the   production   of   autoantibodies   in   amounts

sufficient to cause thrombocytopenia. 

23
 Figure 1 gives an explanation about the trigger factors for autoantibody
 
production, which to this date are still unknown. In the initial stage, glycoproteins

IIb/IIIa   are   recognized   by   autoantibodies,   while   antibodies   recognizing

glycoproteins   Ib/IX   have   not   been   formed   at   this   stage.   Autoantibody­coated

platelets will bind to antigen presenting cells (APCs), i.e. macrophages, through

Fc receptors, and are then internalized and degraded. The APCs process not only

glycoproteins IIb/IIIa, but also other glycoproteins. The activated APCs express

new   peptides   on   their   surface,   aided   by   costimulation   (shown   by   interaction

between CD154 and CD40). The new peptides subsequently are presented to T

cells, whereupon these activated T cells produce cytokines and activate B cells to

4  
produce   antibody   against   specific   platelet   glycoproteins. In   addition   to

autoantibody­   mediated   platelet   destruction,   ITP­associated   thrombocytopenia

may also be caused by suboptimal platelet production.5

24
The mechanism of the occurrence of thrombocytopenia in ITP was more complex
than originally thought. Platelet damage in ITP involves autoantibodies against
glycoproteins   found   in   the   platelet   membrane. So   that   there   is   destruction   of
platelets   that   are   covered   with   antibodies   ( antibody­coated   platelets )   by
macrophages   found   in   the   spleen   and   other   reticuloendothelial   organs.
Megacariocytes   in   marrow bone can normal or increase on ITP. While
thrombopoitin   levels   in   plasma   which   are   progenitors   of   proliferation   and
maturation of platelets have decreased significantly, especially in chronic ITP. 6

         

              The existence of clinical and demiological episodes between acute and
chronic   ITP,   suggests   that   there   is   a   difference   in   the   pathophysiological
mechanism   for  the  occurrence   of  thrombocytopenia   between  the   two. In  acute
ITP, it has been believed that platelet destruction is increased due to the presence
of antibodies that are formed when there is an immune response to bacterial / viral
infections or on immunization, which reacts with antigens from platelets. Other
mediators that increase during the occurrence of an immune response to infection

25
can contribute to the suppression of platelet production. In chronic ITP there may
be a disturbance in immune system regulation as in other autoimmune diseases,
which   results   in   the   formation   of   antibodies   specific   to   platelets.     Currently,
several   types   of   platelet   surface   glycoprotein   have   been   identified   in   ITP,
including GP IIb­IIa, GP Ib, and GP V. However, how antithrombocyte antibodies
increase in ITP, the exact difference in acute and chronic ITP pathophysiology, as
well as the components involved in regulation are still unknown. The foregoing
explains why some of the latest treatment methods used in ITP management have
limited effectiveness, because they fail to reach specific targets of immunological
pathways that are responsible for changes in platelet production and destruction. 

2.5 Clinical Manifestations 

Bleeding in ITP is manifested by purpura, ecchymoses and petechiae, and
mucosal hemorrhages. Hemorrhagic vesicles  or bullae may be seen in the oral
cavity and other mucosal surfaces. Gingival bleeding and epistaxis are the most
frequent   types   of   hemorrhage. Other   types   of   bleeding   may   occur   in   the
gastrointestinal tract as melena, and in the genitourinary tract as hematuria and
menorrhagia. Spontaneous   mucosal,   intracranial   and   gastrointestinal   bleeding
occurs at a platelet count of <10,000 / u L. 7

o The signs and symptoms of idiopatic purpura thrombocytosis are
increased   bleeding   due   to   decreased   platelet   count. Form   of
bleeding in:

 Purpura. Bleeding   that   occurs   in   the   skin   and   mucous

membranes   (such   as   in   the   mouth)   which   is   purplish   in
color. Blemishes that have no clear cause.

 Petekie. Red spots on the skin. Sometimes red spots blend
together and may look like a rash. Red spots are bleeding
under the skin

 Bleeding is difficult to stop

 Bleeding from the gums

 Nosebleed

26
  Prolonged menstruation in women

 Hematuria

 Gastrointestinal bleeding

Intracranial   hemorrhage   is   the   most   serious   complication   in   ITP. This

concerns almost 1% of patients with severe thrombocytopenia. Bleeding is usually
subarachnoid, often multiple and varying in size from petechia to extensive blood
extravasation.

2.6 Diagnosis

To date the diagnosis of ITP is still arrived at by a process of exclusion, by

eliminating other causes of thrombocytopenia, such as thrombocytopenia
associated with autoimmune diseases, exposure to drugs such as heparin or
8
quinine, and HIV or hepatitis C infection. Thrombocytopenia associated with
HIV or hepatitis C infection may be clinically indistinguishable from primary
thrombocytopenia and may occur several years before the development of other
9
symptoms. The diagnosis of ITP also requires a medical history (anamnesis),
physical examination, platelet count, and examination of a peripheral blood smear.
The medical history may eliminate other causes of thrombocytopenia such as
drugs or alcohol. On physical examination usually signs of bleeding are
encountered, such as petechiae, purpura, and conjunctival and mucosal

27
hemorrhages (Figure 2). Mild splenomegaly may also be found in young patients.

In general, ITP patients appear healthy, but suddenly experience bleeding

in the skin (petechiae or purpura) or on the nasal mucosa (epistaxis). You should

also look for a history about the use of drugs or other ingredients that can cause

thrombocytopenia.   Family   history   is   generally   not   obtained.     On   physical

examination   there   is   usually   only   evidence   of platelet   type   bleeding ,   namely

petechiae, purpura, conjunctival hemorrhage, or other mucocutaneous bleeding. is

necessary to consider the possibility of another disease, if there is an enlarged

liver and / or spleen, although the tip of the spleen is palpable in approximately

10% of children with ITP. 

Laboratory Findings

EXAMINATION OF SUPPORT

To ensure the diagnosis of Idiopathic Thrombocytopenic Purpura, is done by

the right laboratory examination. Examinations can be carried out, among
others, by examination:

1. Regular blood tests, low platelet values (<150,000) with erythrocytes (if no
heavy bleeding occurs) and leukocytes within normal limits will be obtained.

28
2. Examination of peripheral blood, thrombocytopenia with erythrocytes and
leukocytes with normal morphology will be obtained. Young platelets with a
larger size (megatrombosit) are found.

3. Examination of PT and APTT is within normal limits, normal fibrinogen.

4. Monoclonal antigen capture assay. Platelet measurements are associated

with antibodies, directly to measure platelets associated with antibodies.

5. Normal bone marrow examination or increase in megacariocyte and

agranular counts, and do not contain platelets.

Guidelines from the American Society of Hematology state that bone

marrow examination is not needed at the age of> 40 years, patients with a non-
characteristic picture (cytopenia picture) or patients who do not respond well to
therapy. Although not recommended, many hematological pediatricians
recommend bone marrow examination before starting corticosteroid
administration to rule out cases of acute leukemia. 1

2.6 Management

Management   of   ITP   in   children   includes   supportive   measures   and

pharmacological therapy. Supportive actions are important in the management

of ITP in children, including limiting physical activity, preventing bleeding due

to trauma, avoiding drugs that can reduce platelet production or changing their

functions, and what is important is giving understanding to patients and / or

parents about their illness . Most cases of ITP in children do not need to be

29
hospitalized, because they can recover completely spontaneously in less than 6

months.1,5,11  In some cases ITP in children is found to have permanent skin

bleeding, mucosal bleeding, or internal bleeding life­threatening conditions that

require   immediate   action   or   treatment.5,10  Platelet   transfusion   is   rare   and   is

usually ineffective, because the transfused platelets are immediately destroyed.5

Sudden recurrence is usually rare. In patients whose platelet counts do not

reach   normal   values  in   6   months,   the   diagnosis   changes   to   chronic

ITP.1,5,10,11 Serious bleeding is rarely found in ITP, the incidence of cerebral

hemorrhage in ITP in the first week is only 0.1­0 , 2%, but increases to 1% in

those with a platelet count of less than 20,000 / mm3 after 6­12 months. 3 Brain

bleeding in ITP is not always fatal, and treatment does not reduce the risk of

brain hemorrhage in ITP.

The usual treatment for children with ITP includes oral corticosteroids,

intravenous immunoglobulin (IVIG), and finally, anti­D for rhesus D cases is

positive. The treatment has the potential to have serious side effects, so it is

important for us to consider these risks so as not to harm the patient (primum

non nocere).

Before the IVIG era, oral corticosteroids were the main treatment in ITP

because   they   were   believed   to   inhibit   the   destruction   of   platelets   in   the

reticuloendothelial system and reduce the formation of antibodies to platelets,

and have a capillary stabilizing effect that can reduce bleeding. Buchanan and

Holtkamp (1984) conducted a study of the effectiveness of oral corticosteroids

at standard doses (2 mg / kg / day) as acute ITP treatment. Based on platelet

count,   bleeding   time,   and   clinical   symptoms,   there   was   no   significant

difference between the prednisone and placebo groups except in the 7th day

30
treatment.   Recent   research   shows   a   faster   response   (as   fast   as   IVIG)   in

increasing platelet counts at higher prednisone doses (4 mg / kg / day).

Some studies have shown a rapid increase in platelet counts with minimal

side   effects   on   IVIG   treatment.   Like   cortico­steroids,   IVIG   also   causes   a

blockade of the reticuloendothelial system which can increase platelet counts

quickly (generally in 48 hours), making it the treatment of choice for ITP with

serious (clinically severe) bleeding. Although IVIG has been popularly used in

ITP therapy in children, the latest data shows that more than 75% of children

experience side effects of headache and heat. Some experience more serious

side effects, namely meningeal irritation and transient hemiplegia. Therefore,

IVIG   should   not   be   given   without   a   clear   indication,   especially   if   only   to

increase the platelet count.13

Treatment   with   anti­D   immunoglobulin   is   effective   in   rhesus­positive

children and has the advantage of a single injection in a short time. But besides

being   expensive,   it   is   reported   that   hemolysis   and   anemia   require   blood

transfusion after treatment. There are several studies comparing combinations

of several treatment options including non­therapy, oral prednisone, high­dose

methylprednisolone, IVIG, and intravenous anti­D immunoglobulin.

Imbach,   et   al.   (1985)   conducted   the   first   study   comparing   IVIG   and

steroids. There was no significant difference in response between the two in

children whose platelet counts increased> 30,000 / μl within 10 days, but IVIG

was better in those whose platelets increased> 30,000 // μl within> 10 days.

Albayrak, et al. (1994) compared high­dose methyl­prednisolone (30 mg /

kg / day and 50 mg / kg / day for 7 days) with IVIG (0.5 g / kg / day for 5

doses) for the treatment of acute ITP. There was no significant difference in

31
increasing platelet counts among the three.

The healing process will occur spontaneously in children with ITP, but may be

accelerated   by   the   administration   of   high­dose   corticosteroids   or   IVIG,   the

response   is   often   only   temporary   and   does   not   provide   protection   against

severe bleeding complications that can be life­threatening. There are also no

data showing that the treatment decreases the likelihood of chronic ITP. 5 Long­

term steroid administration should be avoided because the risk of side effects

may   be   more   dangerous   than   the   disease   itself.13  Splenectomy   is   rarely

performed in children with ITP and is only recommended for severe bleeding

that does not respond to treatment, and is carried out after becoming chronic

ITP (> 6 months). The rate of failure of splenectomy ranges from 25­30% and

may be greater (> 60%) with long­term observation. Splenectomy, although

rarely   associated   with   an   increased   risk   of   sepsis   despite   pneumococcal

vaccination and penicillin prophylaxis.3

2.8 Prognosis

Treatment   response   can   reach   50%   ­70%   with   corticosteroids.   Adult   ITP

patients with only a small proportion can experience spontaneous remission of

causes of death in ITP usually caused by intracranial bleeding which has fatal

effects ranging from 2.2% for ages over 40 years and up to 47.8% for ages over

60 years.

32
 CHAPTER III

CASE ANALYSIS

Theory Case
Etiology The underlying cause is unknown,  Unknown

but has many trigger factor
Clinical  ­ Bleeding in ITP is manifested by  ­ Petechiae 

Manifestatio purpura, ecchymoses and petechiae, ­ Haematoma

n and mucosal hemorrhages. 

­ Hemorrhagic vesicles or bullae may

33
 be seen in the oral cavity and other 

mucosal surfaces. 

­ Gingival bleeding and epistaxis are 

the most frequent types of 

hemorrhage. 

­ Other types of bleeding may occur 

in the gastrointestinal tract as 

melena, and in the genitourinary 

tract as hematuria and menorrhagia.

­ Spontaneous mucosal, intracranial 

and gastrointestinal bleeding occur 

at a platelet count of <10,000/ uL.
Diagnosis ­ The diagnosis of ITP also  ­ Patient have low 

requires a medical history  platelet counts and 

(anamnesis), physical  also a sign of 

examination, platelet count,  bleeding such as; 

and examination of a  petechiae on the 

peripheral blood smear. The  head, face and lower

medical history may eliminate extremity and 

other causes of  haematoma on 

thrombocytopenia such as  auricular sinistra.

drugs or alcohol. On physical 

examination usually signs of 

bleeding are encountered, 

such as petechiae, purpura, 

and conjunctival and mucosal 

hemorrhages.

34
Physical examination: On physical  Signs of bleeding like 

examination usually signs of bleeding petechiae is 

are encountered, such as petechiae,  encountered on 

purpura, and conjunctival and  patient’s head, face, 

mucosal hemorrhages. lower extremity and 

chest. (+)
-To ensure the diagnosis of ­ Laboratory 
Idiopathic Thrombocytopenic
evaluation:
Purpura, is done by the right
1. Hematology: 
laboratory examination.
thrombocytopeni
Examinations can be carried out,
among others, by examination: 2. Bleeding time and 
- Regular blood tests, low platelet Clotting 
values (<150,000) with
time:borderline 
erythrocytes (if no heavy bleeding
maximum normal 
occurs) and leukocytes within
range
normal limits will be obtained.
- Examination of peripheral
blood, thrombocytopenia with
erythrocytes and leukocytes with
normal morphology will be
obtained. Young platelets with a
larger size (megatrombosit) are
found.
- Examination of PT and APTT is
within normal limits, normal
fibrinogen.
-Monoclonal antigen capture
assay. Platelet measurements are
associated with antibodies,
directly to measure platelets
associated with antibodies.
Normal bone marrow

35
examination or increase in
megacariocyte and agranular
counts, and do not contain
platelets

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