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English Wikipedia
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lüll Current concepts in thrombotic thrombocytopenic purpura Tsai HMAnnu Rev Med 2006[]; 57 (ä): 419-36Recent advances have demonstrated that thrombotic thrombocytopenic purpura (TTP), characterized by widespread thrombosis in the arterioles and capillaries, is caused by deficiency of a circulating zinc metalloprotease, ADAMTS13. Two types of TTP are recognized: autoimmune TTP, caused by inhibitory antibodies of ADAMTS13, and hereditary TTP, caused by genetic mutations of ADAMTS13. This article reviews the characteristics and function of ADAMTS13, the mechanism by which ADAMTS13 deficiency may lead to thrombosis, and the causes of ADAMTS13 deficiency. It also discusses how the new knowledge may improve the diagnosis and treatment of this previously mysterious disorder.|ADAM Proteins/deficiency/*physiology[MESH]|ADAMTS13 Protein[MESH]|Blood Platelets/physiology[MESH]|Diagnosis, Differential[MESH]|Humans[MESH]|Purpura, Thrombotic Thrombocytopenic/diagnosis/*etiology/therapy[MESH]|Vascular Resistance/physiology[MESH]|von Willebrand Factor/physiology[MESH] |