Natural History of Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome
Natural History of Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome
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DOI：
10.1055/s-0034-1395154
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发表时间：
2014-11-01
影响因子：
5.7
通讯作者：
Yamashita, Yoshiki
中科院分区：
文献类型：
--
作者：
Wada, Hideo;Matsumoto, Takeshi;Yamashita, Yoshiki
随着(1)腹泻相关溶血性尿毒症综合征(HUS)与产志贺毒素大肠杆菌-HUS(STEC-HUS)、(2)ADAMTS-13(一种具有凝血酶反应蛋白1基序的去整合素和金属蛋白酶，成员13)水平显著降低与典型的血栓性血小板减少性紫癜(TTP)以及(3)补体调节系统异常与不典型HUS(AHUS)之间的关系的建立，血栓性微血管病(TMA)的鉴别诊断变得更加清晰。这些TMA包括典型的TTP、其他形式的TMA、STEC-HUS和aHUS。TMA的病理机制在几种形式的TMA中仍有重叠。关于TMA的管理，还确定了对典型TTP使用血浆置换，对TMA使用额外的类固醇疗法，对ADAMTS-13抑制剂效价较高的典型TTP使用利妥昔单抗，以及对aHUS使用eculizumab。尽管在TMA的病理生理学和管理方面仍存在一些问题，但新的发现有望在不久的将来解决这些问题。
The differential diagnosis of thrombotic microangiopathy (TMA) has become clearer following the establishment of the relationships between (1) diarrhea-associated hemolytic uremic syndrome (HUS) and Shiga toxin-producing Escherichia coli-HUS (STEC-HUS), (2) a markedly reduced ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) level and typical thrombotic thrombocytopenic purpura (TTP), and (3) abnormalities in the complement regulatory system and atypical HUS (aHUS). These TMAs include typical TTP, other forms of TMA, STEC-HUS, and aHUS. The pathological mechanisms of TMA still overlap among several forms of TMA. With respect to the management of TMA, the use of plasma exchange (PE) for typical TTP, additional steroid therapy for TMA and rituximab for typical TTP with a high titer of the inhibitor of ADAMTS-13, as well as eculizumab for aHUS, have also been established. Although several issues remain in the pathophysiology and management of TMA, new findings will hopefully resolve these problems in the near future.