Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an accumulation of ultra-large von Willebrand factor molecules in the plasma which, in turn, initiate the formation of microscopic thromboses in small blood vessels. TTP is a medical emergency. Timely diagnosis and urgent and effective management are vital - mortality in those untreated is in the region of 90%. The understanding of TTP pathogenesis has increased markedly in recent decades. It is now known that TTP is acquired (immunemediated) or...
Thrombotic thrombocytopenic purpura (TTP) is a rare disorder of the blood coagulation system. In most cases, a lack of the ADAMTS13 enzyme leads to an...