Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder caused by the immune-mediated destruction of platelets. It most commonly presents in children between 1-7 years of age, usually after a viral infection. ITP results in low platelet counts and spontaneous bruising or bleeding. Diagnosis involves low platelet count, absence of other abnormalities, and normal bone marrow with increased or normal megakaryocytes. Treatment depends on if it is acute or chronic ITP, and may include corticosteroids, IVIG, anti-D immunoglobulin, thrombopoietin receptor agonists, or splenectomy in severe cases.

1. IDIOPATHIC
THROMBOCYTOPENIC
PURPURA
DR.T.ARIVAZHAGAN
POST GRADUATE
DEPARTMENT OF PATHOLOGY

2. THROMBOCYTOPENIA
•Isolated thrombocytopenia is associated with a
bleeding tendency and normal coagulation tests.
•Generally < 150000
•Spontaneous bleeding 20000 to 50000
•Bleeding from small vessels < 20000

3. RESULTS FROM
•Increased peripheral destruction of
platelets
•Decreased production of platelets in
bone marrow
•Dilutional thrombocytopenia

5. INTRODUCTION
•Also known as Immune Thrombocytopenic Purpura
•A bleeding disorder that involves immunologically
mediated
destruction of the platelets
•Commonest bleeding disorder presenting in children
•Usually presents between 1 and 7 yr of age
•Acute ITP: Thrombocytopenia <6 months- children's
Self imitated , after viral infections

6. ETIO-PATHOGENESIS
Acute ITP Chronic ITP
Viral antigens & viral antibodies
binds to Fc receptors on platelet
Immune destruction of platelets
by macrophages
Autoantibodies predominantly
IgG type
Antibodies direct against specific
platelet glycoproteins GpIIb/IIIa
or Gp1b/IX
Antibody coated platelets
recognized by Fc receptors on
macrophages & destroyed mainly

8. CLINICALFEATURES - SYMPTOMS
• Sudden appearance of bruises and mucosal bleeding
• Epistaxis
• Menorrhagia
• Prolonged bleeds with superficial trauma
• Symptoms appear often 1-2 weeks after a self-limited viral illness,
• Purpuric spots
• Ecchymosis
• Hematuria

9. PHYSICALEXAMINATION
•Petechiae, purpura, ecchymosis
•Splenomegaly, lymphadenopathy, bone pain are unusual
and if present, other causes of thrombocytopenia should
be thought of like leukemia

12. Parameters Acute ITP Chronic ITP
Age Childhood Young adults
Sex No sex preference Females > males
H/O preceding
viral infection
Present Absent
Onset of bleeding Sudden Insidious
Degree of
thrombocytopenia
Severe Moderate
Duration of disease Self limited ( 2-
6months)
Many years
Spontaneous
remission
Usual Rare

13. INVESTIGATIONS
•Peripheral blood:
•Blood loss – anemia
•Lymphocyte , eosinophils increased in case of
children's
•Platelet count – reduced < 20000
•Platelets are large in size

15. •Bone marrow :
•Megakaryocytes normal or increased in number
•Hypo granularity of cytoplasm
•Vacuolization
•Lack of platelet budding
•Hypolobulation
•Dense nuclear chromatin

19. •Coagulation profile :
•Prolonged bleeding time
•Platelet antibodies:
•Platelet associated immunoglobulins are
raised

20. DIAGNOSIS
•Mucocutaneous type of bleeding with abrupt
onset
•Other abnormalities on physical examination
•Presence of isolated thrombocytopenia with no
other abnormality on complete blood count
•Bone marrow examination is normal

21. TREATMENT
•Goal of therapy: Minimizing the risk of
hemorrhage and decreasing the long-term
side effects of treatment

22. Acute ITP:
• 1. Close observation for platelet count >20,000/uL
• 2. IVIG 1 g/kg/day for 1-2 days
• 3. Corticosteroids: Prednisone 1-4 mg/kg/day for 2-4 weeks and then tapered
• 4. Intravenous anti-D immunoglobulin 50-75 mg/kg in Rh +ve children
• 5. If severe hemorrhage occurs, platelet transfusion under the cover of steroids
Chronic ITP:
• 1. Low dose Prednisone on alternate days
• 2. Rituximab (anti-CD20 monoclonal antibody)
• 3. Cyclosporine
• 4. Azathioprine
• 5. Thrombopoietin receptor-binding agents (Eltrombopag, Romiplostim)

23. SURGICAL MANAGEMENT: SPLENECTOMY
Indications:
• 1. Child >4 yr, severe ITP for >1 yr i.e. chronic ITP
• 2. Symptoms not controlled with medical therapy
• 3. When life-threatening hemorrhage (intracranial hemorrhage) complicates
• Acute ITP and platelet count cannot be corrected rapidly with transfusion of platelets and
administration of IVIG and corticosteroids
Complications:
• 1. Lifelong risk of overwhelming post-splenectomy infection by encapsulated organisms
• 2. Increased risk of thrombosis
• 3. Potential development of pulmonary HTN in adulthood