This document discusses Immune Thrombocytopenic Purpura (ITP), a blood disorder characterized by low platelet count due to increased platelet destruction by the immune system. ITP is the most common cause of thrombocytopenia in children, affecting those between ages 2-10. Treatment options discussed include corticosteroids, intravenous immunoglobulin, anti-D therapy, splenectomy, rituximab, thrombopoietic agents, and high-dose dexamethasone. Supportive measures and avoiding medications that affect platelets are also recommended.

1. IMMUNE
THROMBOCYTOPENIC
PURPURA
(ITP)
R.Anusha,
PharmD Intern,
170514882007.
1

2. IMMUNE THROMBOCYTOPENIC PURPURA
(ITP)
 The most common(4-8 /100,000) cause of
thrombocytopenia in children and a result of
immunologically mediated increased platelet
destruction.
 Acute and self-limiting but may be recurrent or
chronic disorder.
 Mainly affects children between 2 and 10 years
of age.
 Boys and girls are equally affected.
2

3.  Idiopathic thrombocytopenia purpura is a blood disorder
affecting the platelets.
 It is a condition in, which there is bruising (purpura)
because there are fewer platelets in the blood than
usual (thrombocytopenia) and it is not known what
causes it (idiopathic).

3

4.  The condition referred to as ‘immune’
thrombocytopenia purpura which means it could have
been caused by something going wrong with the immune
system (the body’s defence against infection) or an
allergic reaction
4

5. Two Types of ITP
 Acute Immune Thrombocytopenic Purpura
The course of disease is shorter or equal to than 6
months.
 Chronic Immune Thrombocytopenic Purpura
The course of disease is longer than 6 months.
5

6. ETIOLOGY
 Idiopathic.
 Exact antigenic target for most such antibodies in most
cases of childhood acute ITP remains undetermined.
 In chronic ITP May demonstrate antibodies against
platelet glycoprotein.
 Most common viruses which are associated with ITP are
EBV (short duration) & HIV (chronic).Rarely in children
infected with H.pylori.
 These viral infections trigger the body to produce
antibodies to destroy the virus. It is thought that these
antibodies target platelets sothe spleen and liver see them
as foreign and destroy them.
6

7. PATHOGENESIS
7
 An autoantibody directed against the platelet surface
develops with resultant sudden onset of
thrombocytopenia.
 After binding of the antibody to the platelet surface,
circulating antibody-coated platelets are recognised by
the Fc receptors on splenic macrophages, ingested and
destroyed.

8. 8

9. 9

10. SIGNS AND SYMPTOMS
 Scattered petechiae
 Bleeding signs and symptoms
 Mucosal bleeding
 GI Bleeding
 Intracranial hemorrhage
 Hematuria
 Thrombocytopenia
 Purpura
10

11.  Excessive bleeding with minor injuries
 Spontaneous bleeding from the mouth and nose
 Unexplainable or spontaneous bruising
 Excessive internal bleeding
 Disturbed sleep cycle/ Insomnia
 Irregular appetite
 Depression
11

12. 12

13. DIAGNOSTIC TESTS
 Coagulation test : PT and PTT are normal
 Bleeding time: Prolonged
 CBC: Low number of platelets
 Platelet associated Antibody may be detected
 Bone Marrow Examination: The bone marrow in
patients with ITP contains normal or increased
numbers of megakaryocytes, Indicating that platelet
production is normal and that thrombocytopenia results
from increased platelet destruction.
 NO SPECIFIC TEST THAT IS DIAGNOSTIC OF
ACUTE OR CHRONIC ITP
13

14. TREATMENT
 There are NO DATA showing that treatment affects
either short term or long term clinical outcome of
ITP.
SUPPORTIVE MEASURES:
 Reassurance.
 Restricting physical activity and avoiding trauma.
 Avoid medications that suppress platelet production
or alter their function e.g, aspirin, heparin, valproic
acid, digoxin etc.
14

15. INTRAVENOUS IMMUNOGLOBULIN(IVIG):
 MECHANISM:Blocking Fc receptor of the RE (reticulo-
endothelium) phagocytes. Preventing them from binding
and destroying IgG antibody-coated platelets.
 DOSE:As per American Society of Hematology
Guidelines: “A single dose of IVIG (0.8-1g/kg) or short
course of corticosteroids should be used as first line
treatment”. Dose of 0.8 – 1g/kg/day for 1-2 days induces a
rapid rise in platelets usually > 20 ˣ109 /L in 95% patient
within 48hours.
 MERIT:Results in faster elevation of the platelet count and
may be indicated in severe disease.
 LIMITATION OF IVIG: Expensive, high frequency of
headaches and vomiting suggestive of IVIG-induced
aseptic meningitis.
15

16. CORTICOSTEROIDS:
 MECHANISM:Have a rapid, dose-dependent action
that reduce RE destruction of antibody-coated
platelets. Also more slowly reduces antibody
production.
 DOSE:Oral prednisone 2 to 4 mg/kg/d tapered over 2
to 4 weeks. Children with chronic ITP who have mild
or recurrent bleeding are sometimes treated with
intermittent courses of IVIG or high dose
corticosteroids(intravenous methylprednisolone
20~30mg/kg/d for 3 days, then decrement).
 MERIT:Cheap and convenient.
 DISADVANTAGE:Long - term use of corticosteroids
should be avoided because of potential
toxicity(moodiness,weight gain, etc)
16

17. IV ANTI-D THERAPY:
For Rh +ve patients.
Ineffective in Rh –ve patients.
MECHANISM: Anti- Rh(D) immunoglobulin produces a
mild hemolytic anemia that saturates the Fc receptors of the
phagocytic elements of the RE system. Permitting increased
survival of antibody-coated platelets.
DOSE:50-75µg/kg causes rise in platelet count to > 20 ˣ109
/L in 80-90% of patients within 48-72hours.
MERIT : less expensive than IVIG but more costly than
steroids,lower rate of allergic side effects(10%) than IVIG
and does not cause aseptic meningitis.
DISADVANTAGE:Cause mild hemolysis with a transient
hemoglobin decrease of 10 to 20g/L. 17

18. PLATELET TRANSFUSION:
INDICATION: Platelets are less than 3 x 10 9 /L ,
intravenous platelet infusion is necessary to prevent
severe hemorrhagic tendency.
Platelet infusions have no role except in life -
threatening emergencies.
Because there are a lot of PAIgGs in circulation of
patients, infusion platelets will be destroyed quickly.
18

19. SPLENECTOMY: Should be reserved for 1 of 2
circumstances.
1) The older child (≥4yr) with severe ITP that has lasted >1yr
(chronic ITP).
2) Whose symptoms are not easily controlled with therapy is
a candidate for splenectomy.
Splenectomy must also be considered when life-threatening
hemorrhage (intracranial hemorrhage) complicates acute ITP,
if platelet count cannot be corrected rapidly with transfusion
of platelets and administration of IVIG and corticosteroids.
19

20. ALTERNATIVE TO SPLENECTOMY:
Rituximab used (off-label) in children to treat chronic
ITP. In 30-40% it has induced a partial or complete
remission.
THROMBOPOIETIC AGENTS:Stimulate
thrombopoiesis (in chronic ITP):
1) Romiplastin 2) Eltrombopag
These have FDA approval in adults with chronic ITP
who have insufficient response to
corticosteroids,immunoglobulins,or splenectomy.
20

21. HIGH DOSE DEXAMETHASONE:
Dexamethasone has FDA approval for treatment
of ITP in adults.
21

22. 22

23. 23

24. REFERENCE:
2011 Clinical Practice Guideline on the Evaluation and
Management of Immune Thrombocytopenia(ITP);The
American Society of Hematology.
Raj AB (2017);Immune Thrombocytopenia:Pathogenesis
and Treatment Approaches;Journal of Hematology and
Transfusion,5(1):1056.
24