Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura.
Advantages and limits of ADAMTS13 testing in the prognostic assessment of thrombotic thrombocytopenic purpura.
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DOI：
10.1016/j.lpm.2011.10.025
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发表时间：
2012-03-01
期刊：
影响因子：
--
通讯作者：
Franchini, Massimo
中科院分区：
文献类型：
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作者：
Mannucci, Pier Mannuccio;Franchini, Massimo
血栓性血小板减少性紫癜(TTP)是一种罕见但严重的疾病，以机械性溶血性贫血和消耗性血小板减少为特征，导致弥漫性微血管血栓形成，导致器官缺血和功能损害的体征和症状。在阐明了TTP的病理生理机制后，由于证实了先天或后天(自身免疫)血浆中von Willebrand因子裂解金属蛋白A去整合素和金属蛋白酶与凝血酶反应蛋白1重复序列(ADAMTS13)的缺乏症，许多实验室检测ADAMTS13和相关自身抗体的方法已经发展起来。本文就ADAMTS13和抗ADAMTS13检测的诊断和预后价值的研究进展作一综述。
Thrombotic thrombocytopenic purpura (TTP) is a rare but severe disease characterized by mechanical hemolytic anemia and consumptive thrombocytopenia leading to disseminated microvascular thrombosis that causes signs and symptoms of organ ischemia and functional damage. After the elucidation of the pathophysiology of TTP, thanks to the demonstration of the congenital or acquired (autoimmune) plasma deficiency of the von Willebrand factor cleaving metalloprotease A Disintegrin And Metalloprotease with ThromboSpondin 1 repeats (ADAMTS13), a number of laboratory assays for measuring ADAMTS13 and related autoantibodies have been developed. Current knowledge on the diagnostic and prognostic value of ADAMTS13 and anti-ADAMTS13 assays is summarized in this review.