ILA : IDIOPATHIC /IMMUNE THROMBOCYTOPENIC PURPURA (ITP)/PURPURA HEMORRHAGICA /ESSENTIAL THROMBOCYTOPENIA.

What is ITP?Bleeding disorder where the blood doesn't clot as it should due to low number or platelet

• Immune : there is autoimmune antibodies in their blood• Thrombocytopenic : thrombocyte/platelet count below 100,000 per microliter.

•Purpura :purplish or reddish-brown skin rash caused by the leakage of blood from broken capillaries into the skin.

PATHOGENESIS

CLINICAL PICTURE Acute chronic

85-90% cases 10 % cases

Self limited within 6 months (platelet return to normal)

Thrombocytopenia > 6 months

Mostly children 2-8 years

History of viral infection in preceding 1-4 weeks

CLINICAL PICTURE – Acute

DIFFERENTIAL DIAGNOSIS OF ITP•Disseminated Intravascular Coagulation•Thrombotic Thrombocytopenic Purpura•Myelodysplastic Syndrome•Acute leukemia•Drug-induced thrombocytopenia•Hemolytic-uremic syndrome (HUS)•Human immunodeficiency virus (HIV)•Hypersplenism•Liver disease•Lymphoma•Malignancy•Posttransfusion reaction•Pregnancy induced thrombocytopenia•Sepsis•Systemic lupus erythematosus (SLE)

Investigations 1. CBC:• Isolated thrombocytopenia: Always <100,000 and often <20,000/mm3

• Normocytic normochromic anemia only if due to significant bleeding

2. Bleeding time is prolonged (normal: 2-5 minutes)

3. Coagulation time (4-8 min), prothrombin time and partial thromboplastin time is normal

4. Clot retraction: Poor or absent

5. Bone marrow examination: • Megakaryocytes: Normal or increase number with decrease or absent budding• Granulocytic and erythrocytic series are normal

4. ANA in adolescents (to rule out SLE)

5. Coombs’ test if there is anemia

6. Test for HCV and HIV for the newly diagnosed adult patients with ITP

TREATMENT OF ITPMild case : platelets more than 30,000/mm3-mild bruising- No treatment (only observation) except preparation for surgery.- Avoid trauma and violent sport.

Severe case : platelet less than 20,000/mm3-marked bruising-mucous membrane bleeding-CNS bleeding

1st line therapy• Oral corticosteroids (1mg/kg weight of patient)- Response to 2/3 of the patients.- Relapse is usual when the dose is reduced.

• Intravenous immunoglobulin (IgG)-give especially before surgery-rapid rise in desired platelet count.

2nd line therapy• Splenectomy (last resort) – give respond to majority of cases- Indications 1) Emergency in acute severe ITP (ie : intracranial haemorrhage)2) Chronic ITP not respond to medical treatment

If fail :3) High dose corticosteroids4) IV anti D for Rh+5) Vinca alkaloids 6) Danazol7) Immunosuppresive drugs (azothiopine,cyclosporine,dapsone)8) Thrombopoiesis protein( Rituximab,Romiplostin)