Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adults
Diagnostic and risk criteria for HSCT-associated thrombotic microangiopathy: a study in children and young adults
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DOI：
10.1182/blood-2014-03-564997
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发表时间：
2014-07-24
期刊：
影响因子：
20.3
通讯作者：
Laskin, Benjamin L.
中科院分区：
文献类型：
--
作者：
Jodele, Sonata;Davies, Stella M.;Laskin, Benjamin L.
移植相关血栓性微血管病（TMA）导致全身性内皮功能障碍，可进展为多器官损伤，严重病例与造血干细胞移植（HSCT）后的不良结局相关。确定严重疾病风险最高的患者具有挑战性。我们前瞻性地评估了100例连续HSCT受者，以确定中度和重度TMA的发生率以及与不良总体结局相关的因素。39例受试者（39%）符合先前发表的TMA标准。TMA受试者的非复发死亡率显著较高（43.6% vs 7.8%，P30 mg/dL），TMA诊断时血液中终末补体激活（sC 5 b-9升高）的证据与极差的生存率相关（
Transplant-associated thrombotic microangiopathy (TMA) leads to generalized endothelial dysfunction that can progress to multiorgan injury, and severe cases are associated with poor outcomes after hematopoietic stem cell transplantation (HSCT). Identifying patients at highest risk for severe disease is challenging. We prospectively evaluated 100 consecutive HSCT recipients to determine the incidence of moderate and severe TMA and factors associated with poor overall outcomes. Thirty-nine subjects (39%) met previously published criteria for TMA. Subjects with TMA had a significantly higher nonrelapse mortality (43.6% vs 7.8%, P30 mg/dL) and evidence of terminal complement activation (elevated sC5b-9) in the blood at the time of TMA diagnosis were associated with very poor survival (