血栓性血小板减少性紫癜：一种由ADAMTS13缺乏引起的血栓性疾病。
Thrombotic thrombocytopenic purpura: a thrombotic disorder caused by ADAMTS13 deficiency.
作者信息
Tsai Han-Mou
机构信息
Division of Hematology, Albert Einstein College of Medicine and Montefiore Medical Center, 111 East 210th Street, Bronx, NY 10467, USA.
出版信息
Hematol Oncol Clin North Am. 2007 Aug;21(4):609-32, v. doi: 10.1016/j.hoc.2007.06.003.
Abstract
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.
摘要
血栓性血小板减少性紫癜（TTP）是一种严重疾病，其特征为微血管血栓形成，累及大脑和其他器官，通常表现为血小板减少、血涂片上出现裂体细胞的溶血现象，以及精神改变或癫痫发作。如果不立即用血浆治疗，患者可能会迅速走向致命结局。最近的研究进展表明，TTP是由循环中的血管性血友病因子裂解金属蛋白酶ADAMTS13缺乏引起的。这一新知识将为改善这种引人关注的疾病的诊断和管理提供线索。
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