Clark William F
Department of Medicine, University of Western Ontario, London, Ontario, Canada.
Semin Dial. 2012 Mar-Apr;25(2):214-9. doi: 10.1111/j.1525-139X.2011.01035.x. Epub 2012 Feb 7.
The classification of thrombotic microangiopathy has evolved and expanded due to treatment and mechanistic advances. The two basic clinical forms of thrombotic microangiopathy (excluding disseminated intravascular coagulation [DIC]), thrombotic thrombocytopenic purpura (TTP), and hemolytic uremic syndrome (HUS) encompass a wide range of primary and secondary forms. The advent of plasma therapy and the identification of an inhibitor to ADAMTS13 in the idiopathic or acute forms of TTP and its absence in diarrheal HUS have had a major impact on our current classification of thrombotic microangiopathy. In adults, the difficulty of differentiating TTP, which is much more common than HUS and the need for a speedy diagnosis to provide life-saving plasma therapy has resulted in the term TTP/HUS for adult forms of thrombotic microangiopathy that present with unexplained thrombocytopenia and microangiopathic hemolytic anemia without a DIC. In this adult population a primary idiopathic and hereditary form as well as eight known secondary categories or clinical forms of TTP/HUS have been identified. HUS also embraces a primary (atypical HUS) and secondary forms (majority, diarrheal HUS secondary to Escherichia coli 0157:H7). In children, who present with HUS with no preceding history of diarrhea, plasma therapy is also offered on an urgent basis and studies are carried out to determine if they are suffering an abnormality in complement activation that may require eculizumab therapy. The advent of plasma therapy in the treatment of thrombotic microangiopathy has led to a clearer understanding of the role of ADAMTS13, both short- and long-term outcomes and the need for future surveillance and intervention.
由于治疗方法和发病机制的进展，血栓性微血管病的分类不断演变和扩展。血栓性微血管病的两种基本临床形式（不包括弥散性血管内凝血 [DIC]），即血栓性血小板减少性紫癜 (TTP) 和溶血性尿毒症综合征 (HUS)，涵盖了广泛的原发性和继发性形式。血浆疗法的出现以及在特发性或急性形式的 TTP 中发现 ADAMTS13 抑制剂，而在腹泻型 HUS 中未发现该抑制剂，这对我们目前血栓性微血管病的分类产生了重大影响。在成人中，区分比 HUS 更常见的 TTP 存在困难，且需要快速诊断以提供挽救生命的血浆疗法，这导致了“TTP/HUS”这一术语的出现，用于描述出现无法解释的血小板减少和微血管病性溶血性贫血且无 DIC 的成人形式的血栓性微血管病。在这一成人人群中，已确定了一种原发性特发性和遗传性形式以及八种已知的继发性 TTP/HUS 类别或临床形式。HUS 也包括原发性（非典型 HUS）和继发性形式（大多数为继发于大肠杆菌 0157:H7 的腹泻型 HUS）。对于没有腹泻前驱史的儿童 HUS 患者，也会紧急提供血浆疗法，并进行研究以确定他们是否存在补体激活异常，这可能需要使用依库珠单抗进行治疗。血浆疗法在血栓性微血管病治疗中的出现，使人们对 ADAMTS13 的作用、短期和长期结果以及未来监测和干预的必要性有了更清晰的认识。
