Immune thrombocytopenia

 immune destruction of platelets → isolated thrombocytopenia

 Primary ITP vs secondary ITP
 Primary — autoantibody against platelets (most common =anti-Gp
IIb/IIIa)
 Secondary — infections, autoimmune diseases,lymphoproliferative
disorders, solid tumors, drugs,miscellaneous

Causes of secondary ITP

 Antiphospholipid syndrome
 Autoimmune: Evan’s syndrome, SLE
 Drug-induced
 HIV, HCV, H.pylori, VZV
 Lymphoproliferative disorders
 Vaccination: MMR

Diagnosis of Primary ITP

 Isolated thrombocytopenia without other cause
 Exclude other causes of thrombocytopenia as below:
 PBS → pseudothrombocytopenia, MAHA, myelophthisis,
blast/abnormal cells, normal platelet morphology
 Drugs
 Splenomegaly, liver disease
 Infection: sepsis, HIV, HCV, malaria, dengue
 Autoimmune

 Bone marrow is NOT routinely required

Basic Laboratory Evaluation
 CBC
 Peripheral blood smear
 Liver function test
 Anti-HIV
 Anti-HCV
 ANA
 Blood group (Rh)
 Reticulocyte count
 Direct Coombs test
 Antiphospholipid antibody
 Bone marrow examination (Selected cases)

Treatment of ITP:
 Goals: control bleeding and avoid treatment-related complication.
 Treat when Platelet count <30×109/L or bleeding

Medications:
1. Corticosteroid
a. Prednisolone 1 mg/kg/day or
b. Dexamethasone 40 mg/d × 4 days
2. Intravenous immunoglobulin (IVIG)
a. Major bleeding, rapid rising of platelets but expensive, temporary
response
b. 1 g/kg/day × 2 day
3. Anti-D Not widely used in Thailand

Response to treatment:
 Complete response: Platelet ≥100,000/μL and no bleeding
 Partial response: Platelet ≥30,000/μL and ↑≥2X and no bleeding
 No response: Platelet <30,000/μL or ↑<2X or bleeding
Second line treatment of ITP
1. Splenectomy: Response 88% (CR 66%), Sx complication 13%, mortality
1%
2. Rituximab 375 mg/m2 weekly × 4, response 50-60%, TTR ~5 wk
3. Thrombopoietic agents: Romiplostim, eltrombopag, TTR 1-2 wk,
response 70-80%, not durable
4. Immunosuppressive agents: cyclophosphamide, cyclosporin A, MMF,
vincristine
5. Colchicine 1.2 mg/d, response 29%
6. Dapsone : 75-100 mg/d, response 50%
7. Danazol 600 mg/d, weak androgen, response 62.7%