Name
THROMBOTIC THROMBOCYTOPENIC PURPURA
DESCRIPTION
DETAIL
CAUSES : 1. DECREASED MARROW PRODUCTION OF MEGAKARYOCYTES - MARROW INFILTRATION WITH TUMOR, FIBROSIS - MARROW FAILURE DUE TO APLASTIC, HYPOPLASTIC ANEMIAS, DRUG EFFECTS - RARELY CONGENITAL AMEGAKARYOCYTIC HYPOPLASIA & THROMBOCYTOPENIA WITH ABSENT RADII ( TAR SYNDROME ) 2. SPLENIC SEQUESTRATION OF CIRCULATING PLATELETS - SPLENIC ENLARGEMENT DUE TO TUMOR INFILTRATE - SPLENIC CONGESTION DUE TO PORTAL HYPERTENSION - OTHER CAUSES OF SPLEENIC ENLARGEMENT LIKE LEUKEMIA, LYMPHOMA OR A MYELOPROLIFERATIVE DISORDER 3. INCREASED DESTRUCTION OF CIRCULATING PLATELETS A. NONIMMUNE DESTRUCTION - VASCULAR PROSTHESES - CARDIAC VALVES - DIC - SEPSIS - VASCULITIS OR ABNORMAL VESSELS - FIBRIN THROMBI - HEMOLYTIC UREMIC SYNDROME - THROMBOTIC THROMBOCYTOPENIC PURPURA B. IMMUNE DESTRUCTION - AUTOANTIBODIES TO PLATELET ANTIGENS - DRUG ASSOCIATED ANTIBODIES - CIRCULATING IMMUNE COMPLEXES (SLE, VIRAL AGENTS, BACTERIAL SEPSIS) 4. DRUG INDUCED A. CHEMOTHERAPEUTIC AGENTS : ESPECIALLY CARBOPLATIN, ALKYLATING AGENTS, ANTHRACYCLINES, ANTIMETABOLITES B. ANTIBIOTICS : SULPHONAMIDES, PENICILLINS , CEPAHLOSPORINS C. HEPARINS : HIGHEST INCIDENCE IS WITH UNFRACTIONATED PRODUCTS D. CARDIOVASCULAR AGENTS : THIAZIDE DIURETICS , RARELY ANGIOTENSIN CONVERTING ENZYMES INHIBITORS * PRESENCE OF HEMOLYTIC ANEMIA ( HEMATOCRIT < 30% WITH SCHISTOCYTES ON PERIPHERAL SMEAR) & PLATELETS < 1,00,000 ( 50% WILL HAVE <20,000 ).
TYPENOTES
TREATMENT : Plasmapheresis is the first-line therapy. Plasmapheresis removes platelet-aggregating substances causing TTP and HUS. Treatment is 90% successful with TTP but is less successful with HEMOLYTIC UREMIC SYNDROME. Steroids have been used, often in conjunction with plasmapheresis. However, steroids are less effective than plasmapheresis (25% response rate). Platelet transfusions should be avoided when possible because they can cause a clinical deterioration. Use platelet transfusions only for uncontrolled bleeding or intracranial hemorrhage. Other therapies include immunosuppressive agents (vincristine, azathioprine, cyclosporine), splenectomy for TTP, and hemodialysis for HUS. Premature termination of pregnancy has been associated with relapse. Delivery should be considered only when no response to other therapies occurs.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
ULTRA SOUND WHOLE ABDOMEN - MALE, BONE MARROW ASPIRATION, COMPLETE BLOOD COUNT