What is thrombocytopenia purpura?

Inhibition of vWF-cleaving protease (ADAMTS-13) idiopathic/autoimmune form, lack of enzyme activity prevents breakdown of vWF. Excess circulation multimers of vWF promote adhesion of plt to endothelium, causes formation of microthrombi, leading to thrombocytopenia. secondary/acquired (cancer, pregnancy, bone marrow transplant, immunosuppressants, HIV-1 we get no ADAMTS-13 suppression) Inherited (Upshaw-Schulman syndrome) Classic pentad (fever, thrombocytopenia, microangiopathic hemolytic anemia, renal failure, altered mentation) fatal if not treated, treatment is plasma exchange (remove offending Abs and replaces ADAMTS-13) may use corticosteroids