PDF(Pubmed)
Abstract：
Thrombotic thrombocytopenic purpura (TTP) is a life-threatening, often immune-mediated disease that affects 2-13 persons per million per year. Hemolytic anemia, thrombocytopenia, and end-organ damage due to the formation of microthrombi are characteristic of TTP. ADAMTS13 is a disintegrin, metalloproteinase, cleaving protein of von Willebrand factor (VWF) that processes the VWF multimers to prevent them from interacting with platelets and, in turn, to microvascular thrombosis. Prompt diagnosis of TTP is critical yet challenging. Thrombotic microangiopathies have similar clinical presentation. Measurement of ADAMTS13 activity helps in the differential diagnosis. Less than 10% ADAMTS13 activity is indicative of TTP. Laboratory ADAMTS13 activity assays include incubating the test plasma with the substrate (full-length VWM multimers) and detection with direct or indirect measurement of the cleavage product. The purpose of this study is to examine the diagnostic potential, advantages, and weaknesses of the ADAMTS13 potency in TTP.
摘要：
血栓性血小板减少性紫癜(TTP)是一种危及生命、通常是免疫介导的疾病，每年影响2-13人。溶血性贫血,血小板减少症,和由于微血栓形成引起的终末器官损伤是TTP的特征。ADAMTS13是一种崩解素，金属蛋白酶,vonWillebrand因子（VWF）的裂解蛋白，处理VWF多聚体以防止它们与血小板相互作用，反过来,微血管血栓形成。TTP的及时诊断是关键但具有挑战性的。血栓性微血管病有相似的临床表现。ADAMTS13活性的测量有助于鉴别诊断。小于10%的ADAMTS13活性指示TTP。实验室ADAMTS13活性测定包括将测试血浆与底物（全长VWM多聚体）孵育，并通过直接或间接测量切割产物进行检测。这项研究的目的是检查诊断潜力，优势,以及TTP中ADAMTS13效力的弱点。
