Pennsylvania State University, Milton S. Hershey Medical Center, Hershey, PA, USA.
Semin Thromb Hemost. 2012 Jul;38(5):469-82. doi: 10.1055/s-0032-1306431. Epub 2012 Apr 3.
Thrombotic microangiopathy, or the syndrome of thrombocytopenia and hemolysis with schistocytes on blood smears, has been a subject of uncertainty and intense controversy. The pathogenesis of thrombotic microangiopathy was unknown and no classification of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome was satisfactory. In recent years, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13) deficiency and defective complement regulation have been identified as the two major causes of noninfectious thrombotic microangiopathy. It is now possible to classify thrombotic microangiopathy pathogenetically rather than clinically, and a distinction between diseases and clinical syndromes is emerging. This pathogenesis-based disease classification requires new diagnostic approaches and provides a framework for rational therapeutic designs. This review discusses the new concepts in the pathogenesis, diagnosis, and management of thrombotic microangiopathy, with particular emphasis on the autoimmune causes of ADAMTS-13 deficiency and defective complement regulation.
血栓性微血管病，或伴有血涂片裂片红细胞的血小板减少和溶血性贫血的综合征，一直是一个不确定和激烈争议的主题。血栓性微血管病的发病机制尚不清楚，血栓性血小板减少性紫癜和溶血尿毒综合征的分类也不尽人意。近年来，解整合素金属蛋白酶与血小板反应蛋白 1 型基质金属蛋白酶 13（ADAMTS-13）缺乏和补体调节缺陷已被确定为非传染性血栓性微血管病的两个主要原因。现在可以根据发病机制对血栓性微血管病进行分类，而不是根据临床表现进行分类，疾病和临床综合征之间的区别正在显现。这种基于发病机制的疾病分类需要新的诊断方法，并为合理的治疗设计提供了框架。这篇综述讨论了血栓性微血管病发病机制、诊断和治疗的新概念，特别强调了 ADAMTS-13 缺乏和补体调节缺陷的自身免疫原因。
