Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.
A serious disorder with characteristic microvascular thrombosis involving the brain and other organs, thrombotic thrombocytopenic purpura (TTP) typically presents with thrombocytopenia, hemolysis with schistocytes on blood smears, and mental changes or seizures. It may progress rapidly to a fatal end if the patient is not treated immediately with plasma. Recent advances have shown that TTP is caused by deficiency of a circulating, von Willebrand factor cleaving metalloprotease, ADAMTS13. This new knowledge will provide clues to improve the diagnosis and management of this intriguing disease.
Tsai
Han-Mou
HM
Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA. htsai@montefiore.org
D051722:ADAM Proteins / Q000172:deficiency* / Q000235:genetics / Q000276:immunology; D000071120:ADAMTS13 Protein; D001323:Autoantibodies / Q000097:blood; D019468:Disease Management; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000175:diagnosis / Q000209:etiology* / Q000235:genetics; D013927:Thrombosis / Q000209:etiology; D014841:von Willebrand Factor / Q000737:chemistry
D001323:Autoantibodies; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human