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移植相关血栓性微血管病:诊断挑战与管理策略

Transplant-associated thrombotic microangiopathy: Diagnostic challenges and management strategies.

作者信息

Elemary Mohamed, Sabry Waleed, Seghatchian Jerard, Goubran Hadi

机构信息

Saskatoon Cancer Centre and College of Medicine, University of Saskatchewan, Saskatoon, Canada.

Saskatoon Cancer Centre and College of Medicine, University of Saskatchewan, Saskatoon, Canada.

出版信息

Transfus Apher Sci. 2019 Jun;58(3):347-350. doi: 10.1016/j.transci.2019.04.022. Epub 2019 May 1.

Abstract

Transplant-associated thrombotic microangiopathy (TA-TMA) is one of the early endothelial complications post Hematopoietic Stem Cell Transplant (HSCT). Several mechanisms during HSCT can contribute to systemic capillary endothelial damage which can lead to TA-TMA among other complications as capillary leak syndrome or engraftment syndrome. Early diagnosis of TA-TMA contributes a challenge due to overlapping clinical manifestations and the absence of specific diagnostic criteria. Incidence is greatly variable between 1-76% according to risk factors of patients and the definition used to confirm the diagnosis. The mortality rates in patients who develop severe TA-TMA are in excess of 80%. Early treatment improves the outcome. This review outlines the diagnostic challenges and therapeutic options for TA-TMA.

摘要

移植相关血栓性微血管病(TA-TMA)是造血干细胞移植(HSCT)后早期的内皮并发症之一。HSCT期间的多种机制可导致全身毛细血管内皮损伤,进而引发TA-TMA以及其他并发症,如毛细血管渗漏综合征或植入综合征。由于临床表现重叠且缺乏特异性诊断标准,TA-TMA的早期诊断颇具挑战。根据患者的危险因素和用于确诊的定义,发病率在1%至76%之间波动很大。发生严重TA-TMA的患者死亡率超过80%。早期治疗可改善预后。本综述概述了TA-TMA的诊断挑战和治疗选择。

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