In TTP, all of the following are seen except: March 2011
Ans. C: Thrombocytosis According to its original description, TTP was defined as the pentad of fever, thrombocytopenia, microangiopathic hemolytic anemia, transient neurological deficits, and renal failure. Thrombotic thrombocytopenic purpura/TTP/Moschcowitz syndrome It is a rare disorder cauing extensive microscopic thromboses to form in small blood vessels throughout the body (thrombotic microangiopathy). 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. A rarer form of TTP, called Upshaw-Schulman syndrome, is genetically inherited as a dysfunction of ADAMTS13. If large vWF multimers persist there is tendency for increased coagulation. Red blood cells passing the microscopic clots are subjected to shear stress which damages their membranes, leading to intravascular hemolysis and schistocyte formation. Reduced blood flow due to thrombosis and cellular injury results in end organ damage. Current therapy is based on suppo and plasmapheresis to reduce circulating antibodies against ADAMTS13 and replenish blood levels of the enzyme Classically, the following five features ("pentad") are indicative of TTP: - Neurologic symptoms (fluctuating), such as hallucinations, bizarre behavior, altered mental status, stroke or headaches - Kidney failure - Fever - Thrombocytopenia, leading to bruising or purpura - Microangiopathic hemolytic anemia (anemia, jaundice and a blood film featuring evidence of mechanical fragmentation of red blood cells)
Renal failure
Hemolyic anemia
Thrombocytosis
Neurological features