Contents

IDENTITY............................................................................................................................................2
ANAMNESIS..........................................................................................................................................2
PHYSICAL EXAMINATION...............................................................................................................6
General Status....................................................................................................................................6
Antropometry Status..........................................................................................................................6
Head to Toe Examination...................................................................................................................7
Neurological Examination.................................................................................................................9
Meningeal Sign..............................................................................................................................9
Motoric Examination...................................................................................................................10
Autonom Examination.................................................................................................................11
Laboratory Investigation..............................................................................................................11
FOLLOW UP..................................................................................................................................12
LITERATURE REVIEW.....................................................................................................................19
DEFINITION..................................................................................................................................20
PATHOPHYSIOLOGY.....................................................................................................................20
CLINICAL MANIFESTATIONS....................................................................................................21
DIAGNOSIS....................................................................................................................................22
DIFFERENTIAL DIAGNOSIS.......................................................................................................22
TREATMENT.................................................................................................................................23
COMPLICATION...........................................................................................................................23

REFERENCES....................................................................................................................................24

Identity
Patient
Name

: An. P.A.S

Birth date

: October 1st 2015

Age

: 10 months

Gender

: Male

Adresss

: Jl. Batu Ampar V No.11 Kramat Jati

Nationality

: Indonesia

Religion

: Islam

Date of admission

: July 31st 2016

Date of examination : August 3rd 2016

Name
Age
Job
Nationality
Religion
Education
Address

Father
Mother
Mr.A.T.
Mrs.Y
37 years old
34 years old
Employee
Housewife
Indonesia
Indonesia
Islam
Islam
High school (graduate)
High school (graduate)
Jl. Batu Ampara V No.11 Kramat Jati

Anamnesis
The anamnesis was taken on August 3rd 2016, by alloanamnesis (from patients mother and
grandmother)

Chief complain : Black diarrhea since six days before admission to the hospital.
Additional complain :Pink Urine since three days before admission to the hospital,
Appearance of redspot on the right ear, arms and legs since two days before admission to
the hospital. Ginggival bleeding since one day before admission to the hospital.

History of present illness

A 10 months old boy came to Raden said Sukanto Police Center Hospital
emergency room suffering from black diarrhea since six days before admission
to the hospital. This complains also followed by pink urine since five days
before admission to the hospital, and since two days before admission appeared
redspots on the right ear, arms and legs.
That black diarrhea happened 6 times a day, contained with a little of
waste, mucous, watery, fishy, and amount a half of glass for each bowel. And
then, there were also pale face and faint body. That complains did not follow
with nosebleed, fever, abdominal pain, cough and cold before.
The next day black diarrhea happened, patients mother brought him to RS
Bhakti Yudha, and he had hospitalization for 4 days there. In that hospital,
patient received therapy 2x200cc whole blood transfusion, 3 units of
trombocytes, Omeprazole 2x0,5cc intravena, Carbapenem 500mg. In 4th day of
hospitalization, patient had gingival bleeding so the next day patient referenced
to RS Polri.
On the days of hospital admission, patient condition is compos mentis and
didnt have adding other complains, even less than before, pink urine and
gingival bleeding did not happen anymore, Black diarrhea still appeared 1 time
at the hospital, increasing desire to eat and did not faint.
Previously the child had a story that 2 weeks before hospital admission,
patient got Measles immunization. In the family, there is no member of the
family get suffer from disease like patient.

History of past illness

Pharyngitis
Bronchitis
Pneumonia
Morbili
Pertussis
Varicella
Diphteria
Malaria
Polio
Enteritis
Bacillary dysentry
Amoeba dysentry
Diarrhea
Thyphoid
Worms
Surgery
Brain concussion
Fracture
Drug reaction

Allergic History

The patient didnt have cows milk allergy.

The patient didnt have asthma, allergic rhinitis, and atopic dermatitis.
The patient didnt have allergy to medicine.
The patient didnt have allergy to dust, pollen, etc.

Birth history
Mothers pregnancy history
The mother routinely checked her pregnancy to the hospital. She denied any
problem noted during pregnancy.
Childs birth history
Labor
: hospital
Birth attendants : doctor
Mode of delivery : pervaginam
4

Gestation
Infant state
Birth weight
Body lenght

: 9 months
: healthy
: 2950 grams
: 49 cm

Development history
First dentition : 6 months
Psychomotor development

Head up
Smile
Laughing
Slant
Speech initation
Prone position
Food self
Sitting
Crawling

: 1 month old
: 1 month old
: 1-2 month old
: 2,5 months old
: 5 months old
: 5 months old
: 5 months old
: 6 months old
: 8 months old

Mental status : Normal

Conclusion: Growth factor and development status is still in the normal limits
and was appropriate according to the patients age.

History of eating
Breast milk
: exclusively 6 months
Formula milk
: bebelac
Fruit and vegetables : apple, banana, carrot, brocolli, spinach, potatoes,
papaya

Immunization history
Immunization

Frequency

Time
5

Hepatitis B
Polio
BCG
DPT
Hib
Campak

3 times
4 times
1 time
3 times
3 times
1 time

0,1,6 months old

0,2,4,6 months old
1 month old
2,4,6 months old
2,4,6 months old
9 months old

Family history
There are no significant illnesses or chronic illnesses in the family declared.
History of disease in other family member
There is no one living around their home known for having same condition as
the patient.
Social and economic history
Patient lived at house with size 20 m x10 m together with parents.
There 1 door at the front side,1 toilet near the kitchen and 2
bedrooms.there are 4 windows inside the house.
Hygiene:
The patients mother changes his clothes everyday with clean clothes.
Bed sheets changed every two weeks.

PHYSICAL EXAMINATION (AUGUST 3 rd 2016)

General status

General condition
Awareness
Pulse
Breathing rate
Temperature

: compos mentis
: compos mentis
: 109 x/min,regular, full, strong
: 24 x/min
: 36,80C

Antropometry status

 Weight :8,4 kg
Height :69 cm
Nutritional status based NCHS (National center for health statistic) year 2000
WFA (Weight for age) : 8,4/9,6 x 100% = 87,5 %
HFA (Height for age) :69/73 x100% = 94,5 %
WFA(Weight for age) :8,4/8,6 x 100% = 97,7 %
Conclusion: the patient has good
nutritional status

Head toe examination

Head
Normocephaly, hair (black,normal distribution,not easily removed) sign of
trauma (-), large fontanelle opened, petechiae +
Eyes
Icteric sclera -/-, pale conjuctiva -/-, lacrimation -/-, sunken eyes -/-, pupils
3mm/3mm isokor,direct and indirect light response ++/++.
Ears
Normal shape, no wound, no bleeding, secretion or serumen -/Nose
Normal shape, midline septum, secretion +/+
Mouth

Lips
Mucous
Tongue
Tonsils
Pharynx

: moist
: moist
: no dirty
: T1/T1, no hyperemia
: no hyperemia

Neck
Lymph node enlargement (-),scrofuloderma (-).
Thorax
i.

Inspection : symetric when breathing, no retraction, ictus cordis is not

visible.
ii. Palpation : mass (-), tactile fremitus +/+
iii. Percussion: sonor sound
iv. Auscultation:
1. Cor
: regular S1-S2, murmur (-), gallop (-)
2. Pulmo
:vesicular +/+, wheezing-/-, ronchy -/-

Abdomen
9

i. Inspection

: Convex, epigastric retraction (-), there is no a widening of

the veins, no spider nevi.

ii. Palpation
: supple, liver and spleen not palpable, fluid wave (-),
abdominal mass (-)
iii. Percussion : The entire field of tympanic abdomen, shifting dullness (-)
iv. Auscultation : normal bowel sound, bruit (-)
Vertebra

: There does not appear scoliosis, kyphosis, and lordosis, do

not look any mass along the line of the vertebral

Ekstremities: warm, ptekie (+), capillary refill time < 2 second, edema (-)
Skin
: Good turgor. Redspots occure in right ear, arms, and legs.

Neurological Examination
Meningeal Sign

Motoric Examintaion
Power
Hand
Feet
Tonus
Hand
Feet
Trophy
Hand
Feet
Physiologic Reflex
Upper extrimities
Biceps
Triceps

5 5 5 5/ 5 5 5 5
5 5 5 5/ 5 5 5 5
Normotonus / Normotonus
Normotonus / Normotonus
Normotrophy / Normotrophy
Normotrophy / Normotrophy
+/+
+/+
10

Lower extrimities
Patella
Achilles
Pathologic Reflex
Upper extrimities
Hoffman
Tromner
Lower extrimities
Babinsky
Chaddock
Oppenheim
Gordon
Schaeffer

+/+
+/+
-/-/-/-/-/-/-/-

Clonus
Patella
Achilles

-/-/-

Autonom Examination
Defecation
Urination
Sweating

Normal ( 1-2 times daily )

Normal ( 4-5 times daily )
Normal

Laboratory Investigation
Hematology 30th July 2016
Hematology

Results

Normal Value

Haemoglobin

13,4

13-16 g/dL

Leukocytes

12.700

5,000 10,000/L

Hematocrytes
Trombocytes

38
5.000

40 48 %
150,000 400,000/L

Erythrocytes

4,98

4 5 million/L

Clinic Chemistry 30th July 2016

Clinic Chemistry

Results

Normal Value

Sodium

137

135-145 mmol/l

Potassium

3,8

3,8-5,0 mmol/l

Chloride

104

98-106 mmol/l
11

Complete Hematology 31st July 2016

Hematology

Results

Normal Value

Haemoglobin

11,9

13-16 g/dL

Leukocytes

11.400

5,000 10,000/L

Hematocrytes
Trombocytes

32
18.000

40 48 %
150,000 400,000/L

Differential Count Leucocytes

Basophil

0-1%

Eosinophil

1-3%

Stick

2-6%

Segment

36

50-70%

Lymphocytes

57

20-40%

Monocytes

2-8%

Erithrocytes

10

<15 mm/hour

4,50

million/ul

Sedimentation

Rate

(LED)
Erithrocytes

Serology/Immunology (Anti Dengue IgG-IgM)

IgG

Negative

Negative

IgM

Negative

Negative

WORKING DIAGNOSIS
ITP
MANAGEMENT
-

IVFD KAEN 3B 10 tpm makro

Transfusion TC 4 unit/days
Inj. Cefotaxime 2x400 mg
Inj. Transamine 3x15mg

PROGNOSIS

Quo ad vitam
: dubia ad bonam
Quo ad functionam : dubia ad bonam
Quo ad sanactionam : dubia ad bonam

12

FOLLOW UP
July 31st 2016 - August 4th 2016
July 31st 2016. First day of Hospitalization, 6th Day of Illness
S

Apperance of red spot on the right ear, arms and legs

Watery stool with bloody color (+) 1x
Cough (-), epistaxis (-), bloody gums (-)
Fever (-)

General condition: Compos mentis.

Heart rate
= 118x/min
Respiratory rate = 26x/min
Temperature
= 36C
Cardio : S1/S2, reguler, no murmur, no gallop
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/Skin: rash (+)

ITP

Ringer Lactat 35tpm

Inj. Cefotaxime 2 x 400 mg i.v.
Inj. Transamin 3x15 mg
Transfusi TC 4 unit/days for 3 days (Day I)

Hematology 30th July 2016

Hematology

Results

Normal Value

Haemoglobin

13,4

13-16 g/dL

Leukocytes

12.700

5,000 10,000/L

Hematocrytes
Trombocytes

38
5.000

40 48 %
150,000 400,000/L

Erythrocytes

4,98

4 5 million/L

Clinic Chemistry 30th July 2016

Clinic Chemistry

Results

Normal Value

Sodium

137

135-145 mmol/l

Potassium

3,8

3,8-5,0 mmol/l

Chloride

104

98-106 mmol/l

Complete Hematology 31st July 2016

13

Hematology

Results

Normal Value

Haemoglobin

11,9

13-16 g/dL

Leukocytes

11.400

5,000 10,000/L

Hematocrytes
Trombocytes

32
18.000

40 48 %
150,000 400,000/L

Differential Count Leucocytes

Basophil

0-1%

Eosinophil

1-3%

Stick

2-6%

Segment

36

50-70%

Lymphocytes

57

20-40%

Monocytes

2-8%

Erithrocytes

10

<15 mm/hour

4,50

million/ul

Sedimentation
(LED)
Erithrocytes

Rate

Serology/Immunology (Anti Dengue IgG-IgM)

IgG

Negative

Negative

IgM

Negative

Negative

August 1st 2016. Second day of Hospitalization, 7th Day of Illness

S

Apperance of red spot on the ear, arms and legs

Watery stool with bloody color (-)
Cough (-), epistaxis (-), bloody gums (-)
Fever (-)

General condition: Compos mentis.

Heart rate
= 122x/min
Respiratory rate = 20x/min
Temperature
= 36,2C
Cardio : S1/S2, reguler, no murmur, no gallop
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/Skin: rash (+)
Ghg

A
p

ITP
Ringer Lactat 35tpm
Inj. Cefotaxime 2 x 400 mg i.v.
14

 Inj. Transamin 3x15 mg

Transfusi TC 4 unit/days (Day-II)
Hematology 1st August 2016

Hematology

Results

Normal Value

Haemoglobin

10,0

13-16 g/dL

Leukocytes

10.700

5,000 10,000/L

Hematocrytes
Trombocytes

24
35.000

40 48 %
150,000 400,000/L

Erythrocytes

3,65

4 5 million/L

August 2nd 2016. Third day of Hospitalization, 8th Day of Illness

S

Apperance of red spot on the forehead, arms and legs

Watery stool with bloody color (-)
Cough (-), epistaxis (-), bloody gums (-)
Fever (-)

General condition: Compos mentis.

Heart rate
= 128x/min
Respiratory rate = 24x/min
Temperature
= 36C
Cardio : S1/S2, reguler, no murmur, no gallop
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/Skin: rash (+)
gh

A
P

ITP

Ringer Lactat 35tpm

Inj. Cefotaxime 2 x 400 mg i.v.
Inj. Transamin 3x15 mg
Transfusi TC 4 unit/days (Day-III)

August 3rd 2016. Fourth day of Hospitalization, 8th Day of Illness

S

Apperance of red spot on the right ear, arms and legs

Watery stool with bloody color (-)
Fever (-)

General condition: Compos mentis.

15

Heart rate
= 125x/min
Respiratory rate = 26x/min
Temperature
= 36,4C
Cardio : S1/S2, reguler, no murmur, no gallop
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/Skin: rash (+)
gh
A

ITP
Ringer Lactat 35tpm
Inj. Cefotaxime 2 x 400 mg i.v.
Inj. Transamin 3x15 mg

Complete Hematology 3rd August 2016

Hematology

Results

Normal Value

Haemoglobin

10,3

13-16 g/dL

Leukocytes

8.800

5,000 10,000/L

Hematocrytes
Trombocytes

38
159.000

40 48 %
150,000 400,000/L

Differential Count Leucocytes

Basophil

0-1%

Eosinophil

1-3%

Stick

2-6%

Segment

36

50-70%

Lymphocytes

57

20-40%

Monocytes

2-8%

Erithrocytes

34

<15 mm/hour

Sedimentation Rate
Erithrocytes

3,90

million/ul

August 4th 2016. Fifth day of Hospitalization, 9th Day of Illness

S

Apperance of red spot on the right ear, arms and legs more disappear

General condition: Compos mentis.

Heart rate
= 114x/min

16

Respiratory rate = 25x/min

Temperature
= 36,2C
Cardio : S1/S2, reguler, no murmur, no gallop
Pulmonary : vesiculer +/+, rhonchi -/-, wheezing -/Skin: rash (+)
Ghg
A
P

ITP
Aff infus

17

18

LITERATURE REVIEW

Immune Thrombocytopenic Purpura (ITP)

Definition
Immune thrombocytopenic purpura (ITP), also known as idiopathic thrombocytopenic
purpura, is an immune-mediated acquired disease of adults and children characterized by
transient or persistent decrease of the platelet count and, depending upon the degree of
thrombocytopenia, increased risk of bleeding
ITP (Immune Thrombocytopenic Purpura) is an acquired bleeding disorder in which the
immune system destroys platelets, blood cells that play a pivotal role in primary hemostasis.
Individuals with ITP develop thrombocytopenia with a platelet count below the normal range
generally defined as less than 150,000 cells/mm3.Thrombocytopenia commonly manifests as
a bleeding tendency,including purpura (easy bruising), and petechiae (extravasation of blood
from capillaries into skin and mucous membranes).

Epidemiology
The annual incidence of ITP is about 3 to 8 cases per 100,000 children with a peak in
the two to five year age group. It should be noted thatthis is an underestimate as the
documented numbers are dependent on the development of bleeding symptoms. There is a
slight male predominance; the ratio of male to female is 1.2:1.0. There is some suggestion
that ITP may have seasonal variation; this finding has not be enconfirmed; however, there is a
strong relationship of acute childhood ITP with recent viral illness or immunization.
The overall incidence in adult hood is based on large registry studies with an estimate
of 100 per million. The incidence in adult males and females is approximately equal except in
the 30 to 60 year age subgroup where the prevalence in females exceeds that of males. To
date, there are no known ethnicities or endemic areas in which ITP is more prevalent.

19

Pathophysiology
The underlying pathologic process resulting in ITP is the generation of autoantibodies
that react with platelet surface antigens. Once bound to the platelets, these autoantibodies
cause platelets to be removed from circulation through phagocytosis via the
reticuloendothelial system, primarily the spleen. The resulting shortened platelet life span
leads to thrombocytopenia; the level of thrombocytopenia observed is based upon each
affected individual's balance between the quantity of antibody produced, the rate of platelet
removal, and the bone marrow's compensatory ability to produce platelets from
megacaryocytes.
ITP is a complex disorder of immune dysregulation; however, the
final pathway is loss of tolerance of the immune system to self-antigens
located on the surface of the platelets and megakaryocytes. Table 1
outlines the different roles that both T and B cells have been speculated to
play in the development of ITP. Fundamentally, ITP results from
antiplatelet antibodies produced by B cells, often targeting primary
platelet glycoproteins such as GP IIb/IIIa.9 Beyond the effects on
circulating platelets, these antibodies are also directed against platelet
glycoproteins on the surface of megakaryocytes, inducing apoptosis-like
programmed
cell death and reducing platelet production. A proposed model for ITP
involves antigen-presenting cells (APCs), which serve the primary function
of internalizing and breaking
down antigenic proteins into smaller peptides. These peptides are then
presented to T cells and, through signaling events, the T cell becomes
activated.Perhaps in certain settings such as inflammation, APCs create
cryptic epitopes that are capable of escaping negative selection. Following
activation, T cells have also been shown to demonstrate alterations in
patients with ITP. Early studies indicated that patients with ITP had
autoreactive T cells that secreted IL-2 upon stimulation with autologous
platelets in an uncontrolled manner. Furthermore,
the T cells observed are primarily against cryptic rather than native
epitopes, supporting a role for APCs as critical cells in the development of
20

ITP. In addition, patients with ITP demonstrate an increased Th1/Th2 ratio

favoring autoreactive B-cell development.

Proposed mechanism of immune dysregulation in ITP.

(A) T cells are activated upon recognition of platelet-specific antigens on the APCs and
therefore induce antigen-specific expansion of B cells. The B cells in turn produce
autoantibodies with specificity for glycoproteins expressed on platelets and megakaryocytes.
(B) Circulating platelets bound by autoantibody are removed by Fc receptors predominantly
by splenic macrophages.
(C) Autoantibodies also reduce the capacity of megakaryocytes to produce platelets.

21

Immunologic mechanisms
The most common mechanism involved in ITP is development of antiplatelet
antibodies through the activation of B-lymphocytes. These antibodies are most frequently
directed against platelet glycoproteins, such as glycoprotein IIb/IIIa (the fibrinogen receptor).
Some antibodies can affect the earlier lineage megakaryocytes and impair their
production of platelets in the bone marrow. Present serological evaluations reveal detection of
antibodies in only 50% of patients and are therefore limited as a laboratory confirmation for
this entity. It is now becoming recognized that cytotoxic T-lymphocytes are also involved in
the pathophysiology of ITP. Therefore, ITP pathogenesis involves a complex network of
systemic events including interaction between B- and T-lymphocytes and inflammatory
cytokines.

Infectious triggers
In the pediatric age group the temporal relationship between development of acute ITP
and a recent (within 2 to 3 weeks) infectious illness or immunization is quite striking and is
reported for approximately 60% of cases.2 Reports from Japan and Italy describe an
association between Helicobacter pylori (H. pylori) infection and ITP. H.pylori is a gramnegative bacterium colonizing approximately 50% of the population. Japanese reports
suggested that concomitant infection with H. pylori may be a causative agent in development
of ITP. Eradication of H. pylori in patients in Japan has led to durable remissions.However,
this finding has not been duplicated in studies in the United States. Therefore, at this time
there does not appear to be a role for routine H. pylori testing in a patient with ITP.
The associations between pathogens, such as H. pylori, and ITP may be related to
molecular mimicry, where the production of antibodies to the pathogen causes the production
22

of antibodies to self. Platelet eluates from patients who were both H. pylori-negative and H.
pylori-positive recognise H. pylori cytotoxin-associated gene A (CagA) protein (Takahashi et
al, 2004). The same study also found that in some patients whose platelet count responded to
H. pylori eradication, levels of anti- CagA antibody in platelet eluates declined, thus
suggesting that molecular mimicry may occur in the pathogenesis of at least

Risk of Immune Thrombocytopenic Purpura After Vaccines

The risk of ITP after vaccination by vaccine and age group is shown in Table down
below. None of the routine childhood vaccines given in the first year of life was significantly
associated with an increased risk of ITP. For vaccines routinely administered at 12 to 19
months of age, there was a significant association of ITP with MMR. For other vaccines
commonly given in this age range (VAR, diphtheria-tetanus-acellular pertussis vaccine
[DTaP], pneumococcal conjugate vaccine [PCV], inactivated poliovirus vaccine [IPV],
Haemophilus influenza type b vaccine [Hib], and HepA), there was no increased risk of ITP
(calculated when not given simultaneously with MMR or MMRV). There Serious adverse
events were defined as having any of the following: intracranial hemorrhage, bleeding
requiring hospitalization, bleeding requiring transfusion of packed red blood cells or platelets,
or death.

23

Classification of ITP
1. Acute ITP
Acute ITP refers to the development of isolated thrombocytopenia with a platelet
count below the normal range (less than 150,000 cells/mm3) and meeting the diagnostic
criteria discussed. The use of the descriptor "acute" refers not to the onset of the disorder, but
rather its duration.ITP that resolves most often in less than 6 months is termed acute.
2. Chronic ITP
ITP is considered chronic ITP by most hematologists if it has persisted greater than 3
months, if it has not responded to a splenectomy and the platelet count has been less than
50,000 cells/mm3. In the pediatric setting, however, the designation for chronic ITP is used
only with duration of disease of 6 months or more.

Clinical Manifestation
Site
Skin

Symptomps
Petechiae,

Mucosal

subcutaneous hematomas
Gingival bleeding, epistaxis, conjunctival
bleeding,

purpura,

menorrhagia,

ecchymoses,

hematuria,

Internal

gastrointestinal hemorrhage
Intracranial hemorrhage, bleeding within

Hemostastic challenges

other organs such as the liver, spleen

Prolonged bleeding after minor surgical
interventions or injury. Bleeding after T&A,
24

menorrhagia,

bleeding

after

dental

extractions, post-partum bleeding

Diagnosis
1. Blood count and evaluation of peripheral smear:
This test rules out involvement of other cell lines such as erythrocytesand white blood
cells. Coexisting anemia may be present in a patient with significant bleeding including
epistaxis or menorrhagia but may also be indicative of the hemolytic anemia of Evan's
syndrome. A review of the blood smear by a trained individual iskey to assure that malignant
disorders such as leukemia, myeloinfiltrative disorders including osteopetrosis, and
microangiopathic disorders such as thrombotic thrombocytopenic purpura (TTP) and
hemolytic uremic syndrome (HUS) are not present.Inherited platelet function disorders such
as Bernard-Souliersyndrome or MYH-9 disorders in which giant platelets are observedmay
also be suspected based upon a review of the blood smear.MYH-9 related disease is
associated with neutrophil inclusions. Additional conditions which may be determined based
upon bloodsmear review include pseudothrombocytopenia, an in vitro artifact caused by
platelet clumping in EDTA anticoagulant and coldagglutinins.
2.Bone marrow evaluation:
If the clinical presentation andreview of the blood smear are typical for ITP, then bone
marrow aspirates and biopsies are often not indicated. The typical finding in a bone marrow
biopsy of a patient with ITP is an increase inmegakaryocytes without other concomitant
abnormalities. Bone marrow aspirate and biopsy are performed based on the clinical
context and would be indicated in the patient with the following features:
a.Atypical clinical symptoms: Presence of malaise,lymphadenopathy, hepatosplenomegaly
or other cytopenias.
b. Age: Many hematologists perform a bone marrow aspirate and biopsy in patients over age
of 60 years (see new recommendations) due to the potential concern for the presence of
25

myelodysplastic syndromes. The most pressing concern for the pediatrician diagnosing ITP is
to ensure that childhood leukemia is not missed prior to initiating treatment with steroids.
Retrospective data from the Pediatric Oncology Group revealed that in approximately 2,000
children presenting with isolated thrombocytopenia, no cases of acute leukemia were present.
c. Refractory ITP: If patients do not respond to therapy appropriately, bone marrow
examination should be performed to exclude other hematological disorders.

3. Blood typing and direct Coombs testing:

Patients with bleeding disorders should have a documented blood type on record and
be informed of these results. Blood typing is also helpful in determining the appropriateness
of certain treatment options such as Anti-D therapy. DAT testing may detect red cell
antibodies seen in Evan's syndrome in which thrombocytopenia is associated with hemolytic
anemia.
4.Immunoglobulin quantitation
Considered in patients before IVIG treatment. These may reveal CVID or selective IgA
deficiency. Therapeutic options for ITP in patients with CVID may be restricted to avoid
profound immunosuppression while patients with selective IgA deficiency may be at risk for
anaphylaxis on exposure to intravenous gamma globulin.

Diferrential Diagnosis

Post tranfussion purpura

PTP is a rare delayed transfusion reaction where a patient develops dramatic,
sudden and self-limiting thrombocytopenia (platelet counts <10 x 109/L in 80% of
cases), typically 7 to 10 days after a blood transfusion. Bleeding from mucous
membranes and the gastrointestinal and urinary tracts is common. Mortality is rare but
may be due to intracranial haemorrhage. Patients usually have a history of
sensitisation by either pregnancy or transfusion with five times more female patients

affected than males.

Disseminated Intravascular Coagulation
Wiskott-Aldich syndrome
Dengue Haemmoragic Fever
Thrombocytopenia-Absent Radius (TAR) syndrome
26

Absence of radii at birth, Platelet count usually 15.000 to 30.000

Neonatal alloimmune thrombocytopenia
Generalized petechiae within first several hours of birth

Treatment
First Line Treatment

Corticosteroid
the first line of therapy for ITP is a corticosteroid, usually prednisone, which can help
raise your platelet count by decreasing the activity of your immune system.
Intravenous Immune Globulin (IVIG).
If you have critical bleeding or need to quickly increase your blood count before
surgery, you may receive medications, such as immune globulin, given intravenously.

Second Line Treatment

Spleenectomy
If prednisone hasn't helped, surgical removal of spleen (splenectomy) may be an
option. This quickly eliminates the main source of platelet destruction in the body and
improves platelet count. However, the cause of thrombocytopaenia remained unclear.
Was the spleen destroying the platelets or did it secrete a suppressive substance that
inhibited platelet production and/ or release into the circulation? Doan et al (1960)
examined a number of spleens from patients with ITP. They demonstrated sea blue
(lipid laden) histiocytes in the spleen, suggesting it was the platelet destroyer. What
directed the spleen to prematurely destroy platelets, however, remained unclear.

(The pathogenesis of immune thrombocytopaenic purpura,bjh review)

Thrombopoietin receptor agonists (TPO-RA)

Most children with immune thrombocytopenia (ITP) will have spontaneous remission
regardless of therapy, while about 20% will go on to have chronic ITP. In those
children with chronic ITP who need treatment, standard therapies for acute ITP may
have adverse effects that complicate their long-term use. Thus, alternative treatment
options are needed for children with chronic ITP. Thrombopoietin receptor agonists
(TPO-RA) have been shown to be safe and efficacious in adults with ITP, and
represent a new treatment option for children with chronic ITP. One TPO-RA,
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eltrombopag, is now approved for children. Clinical trials in children are ongoing and
data are emerging on safety and efficacy.
(Use

of

thrombopoietin

receptor

agonists

in

childhood

immune

thrombocytopenia,13 August 2015)

Rituximab
May be considered for children with ITP who have significant ongoing bleeding
and/or have a need for improved HRQoL despite conventional treatment. May also be
considered as an alternative to splenectomy in children with chronic ITP or as therapy
in those who have failed splenectomy.

Complication
Corticosteroids are accepted as conventional treatment of ITP. However, long term
corticosteroid administration can lead to many undesirable effects. Among them are
hypertension, hyperglycaemia,Cushing's appearance, myopathy and increased susceptibility
to infection.

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REFERENCES
1. Angelica Maria Garzon, William Beau Mitchell,Use of thrombopoietin receptor
2.

agonists in childhood immune thrombocytopenia,mini review: 13 August 2015.

Cindy E. Neunert, Individualized Treatment for Immune Thrombocytopenia:

Predicting Bleeding Risk. 2013; 50:S55S57.

3. Cindy E. Neunert. Current management of immune thrombocytopenia, haematology
2013
4. Provan D, Stasi R, Newland AC, Blanchette VS, Bolton-Maggs P, Bussel JB, et al.
International consensus report on the investigation and management of primary
immune thrombocytopenia. Blood;115:168-86.
5. Wilson D. Acquired platelet defects. In: Nathan D, Orkin S, Look A,Ginsburg D, eds.
Nathan and Oski's Hematology of Infancy and Childhood. 6 ed. Philadelphia: WB
Saunders; :1597-44.
6. Rodeghiero F, Stasi R, Gernsheimer T, Michel M, Provan D, Arnold D, et al.
Standardization of terminology, definitions and outcome of ITP.
7. American Society of Hematology. Guideline on the Evaluation and Management of
Immune Thrombocytopenia (ITP).
8. Nichola Cooper and James Bussel. The pathogenesis of immune thrombocytopaenic
purpura,bjh review. 2012
9. Spring. Immune Thrombocytopenia (ITP): A New Look at an Old Disorder. Indiana
Hemophilia and Trombosis Centre.
10. Department of Pediatrics, University of Colorado Anschutz Medical Campus, Aurora,
Colorado. The Risk of Immune Thrombocytopenic Purpura After Vaccination in
Children and Adolescents. 2011
11.
Behrman RE, Kliegman R, Nelson WE. Hematologi-Onkologi Anak. Jakarta:
Badan Penerbit IDAI; h. 236-. 47. 2.

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