University of Western Ontario, Department of Medicine, London, Ontario, Canada.
Clin J Am Soc Nephrol. 2012 Feb;7(2):342-7. doi: 10.2215/CJN.07230711. Epub 2011 Dec 22.
The classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy-thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and disseminated intravascular coagulation-encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TTP, recognition of the absence of ADAMTS13 inhibition in diarrheal HUS, identification of complement abnormalities in atypical HUS, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from HUS and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy.
由于治疗方法的改进和对与该临床表现相关疾病的认识的提高,血栓性微血管病的分类已经演变和扩展。血栓性微血管病的三种临床形式——血栓性血小板减少性紫癜(TTP)、溶血尿毒综合征(HUS)和弥散性血管内凝血——涵盖了一系列广泛的疾病,可以分为原发性(特发性)或继发性于另一种可识别的疾病或临床情况。在特发性和急性 TTP 中发现了一种对整合素和金属蛋白酶与血小板反应蛋白 1 型基序,成员 13(ADAMTS13)的抑制剂,在腹泻性 HUS 中发现了缺乏 ADAMTS13 抑制,在非典型 HUS 中发现了补体异常,以及对血浆治疗、利妥昔单抗和依库珠单抗治疗作用的更好理解,所有这些都对当前对血栓性微血管病的理解产生了重大影响。在本次主治医生查房中,提出了一名患有血栓性微血管病的患者,并讨论了区分 TTP 与 HUS 和弥散性血管内凝血的困难、快速诊断的必要性以及血浆治疗在适当选择的患者中的作用。讨论试图为成人和儿童患有血栓性微血管病的诊断、治疗选择和未来病程提供一种简单的临床方法。