Thrombotic Thrombocytopenic Purpura is a rare disorder of the blood-coagulation system, causing extensive microscopic thromboses to form in small blood vessels throughout the body.Most cases of TTP arise from inhibition of the enzyme ADAMTS13, a metalloprotease responsible for cleaving large multimers of von Willebrand factor (vWF) into smaller units. Microthrombi formation in the blood vessels lead to Intravascular hemolysis,schistocyte formation and Reduced blood flow due to thrombosis and cellular injury results in end organ damage.
Classically, the following five features are indicative of TTP
* Fluctuating Neurologic symptoms such as hallucinations, bizarre behavior, altered mental status, stroke or headaches
* Kidney failure
* Thrombocytopenia leading to bruising or purpura
* Microangiopathic hemolytic anemia
Due to the high mortality of untreated TTP, a presumptive diagnosis of TTP is made even when only microangiopathic hemolytic anemia and thrombocytopenia is seen, and therapy is started.Current therapy is based on support and plasmapheresis to reduce circulating antibodies against ADAMTS13 and replenish blood levels of the enzyme.