Current concepts in thrombotic thrombocytopenic purpura.
Recent 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.
Tsai
Han-Mou
HM
Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York 10467, USA. htsai@montefiore.org
D051722:ADAM Proteins / Q000172:deficiency / Q000502:physiology*; D000071120:ADAMTS13 Protein; D001792:Blood Platelets / Q000502:physiology; D003937:Diagnosis, Differential; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D014655:Vascular Resistance / Q000502:physiology; D014841:von Willebrand Factor / Q000502:physiology
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human