Current concepts in thrombotic thrombocytopenic purpura

被引：69

作者：

Tsai, HM ^{[1
]}

机构：

[1] Albert Einstein Coll Med, Div Hematol, Bronx, NY 10467 USA

[2] Montefiore Med Ctr, Bronx, NY 10467 USA

来源：

ANNUAL REVIEW OF MEDICINE | 2006年 / 57卷

关键词：

von Willebrand factor; ADAMTS13; shear stress; hemolytic uremic syndrome;

D O I：

10.1146/annurev.med.57.061804.084505

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

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.

引用

页码：419 / 436

页数：20

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