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异基因造血干细胞移植后血栓性微血管病的危险因素及严重后果

Risk factors and severe outcome in thrombotic microangiopathy after allogeneic hematopoietic stem cell transplantation.

作者信息

Uderzo Cornelio, Bonanomi Sonia, Busca Alessandro, Renoldi Mila, Ferrari Pierantonio, Iacobelli Massimo, Morreale Giuseppe, Lanino Edoardo, Annaloro Claudio, Volpe Aldo Della, Alessandrino Paolo, Longoni Daniela, Locatelli Franco, Sangalli Haidi, Rovelli Attilio

机构信息

Clinica Pediatrica dell'Università di Milano-Bicocca, Centro Trapianto di Midollo Osseo, Ospedale San Gerardo, Monza, Italy.

出版信息

Transplantation. 2006 Sep 15;82(5):638-44. doi: 10.1097/01.tp.0000230373.82376.46.

Abstract

BACKGROUND

Thrombotic microangiopathy (TMA) has been described as severe complication after hematopoietic stem cell transplantation (HSCT). The principal aim of this study was to focus the incidence and the outcome of TMA in the era of more complex HSCTs.

METHODS

We analyzed the role of some predicting factors for the incidence and the outcome of TMA after HSCT. We enrolled 539 consecutive patients (307 males, median age 31 years) undergoing HSCT from match or mismatch human leukocyte antigen family donor (314) or match/mismatch unrelated (195) and haploidentical donor (30) for malignant or nonmalignant diseases. TMA diagnosis was performed by homogeneous clinical and laboratory criteria.

RESULTS

Sixty-four of 539 patients presented TMA (11,87%) and the five-year cumulative incidence of TMA was 14% (HR=0.13). Fifty nine of 64 patients were affected by malignant and 5/64 by non-malignant diseases. On multivariate analysis, TMA occurrence was influenced by graft versus host disease >grade II (P=0.0001), donor type (P=0.029), gender (P=0.0233), total body irradiation based conditioning regimen (P=0.0041). Three factors for TMA outcome proved to be statistically significant by multivariate analysis: age (P=0.009), donor type (P=0.0187) and TMA index (P=0.029). The TMA mortality rate was 50%. The outcome was influenced by defibrotide (P=0.02 in univariate analysis).

CONCLUSIONS

The study underlines the possibility of finding out which patients are more prone to developing post-HSCT TMA, and identifies which risk factors are more frequently associated with a dismal outcome after TMA.

摘要

背景

血栓性微血管病(TMA)被描述为造血干细胞移植(HSCT)后的严重并发症。本研究的主要目的是关注在更复杂的HSCT时代TMA的发病率和结局。

方法

我们分析了一些预测因素对HSCT后TMA发病率和结局的作用。我们纳入了539例连续接受HSCT的患者(307例男性,中位年龄31岁),供者为匹配或不匹配的人类白细胞抗原家族供者(314例)、匹配/不匹配的无关供者(195例)或单倍体相合供者(30例),所患疾病为恶性或非恶性疾病。TMA的诊断依据统一的临床和实验室标准。

结果

539例患者中有64例出现TMA(11.87%),TMA的五年累积发病率为14%(风险比=0.13)。64例患者中59例患有恶性疾病,5例患有非恶性疾病。多因素分析显示,TMA的发生受移植物抗宿主病>Ⅱ级(P=0.0001)、供者类型(P=0.029)、性别(P=0.0233)、基于全身照射的预处理方案(P=0.0041)影响。多因素分析证明,TMA结局的三个因素具有统计学意义:年龄(P=0.009)、供者类型(P=0.0187)和TMA指数(P=0.029)。TMA死亡率为50%。结局受去纤苷影响(单因素分析中P=0.02)。

结论

该研究强调了找出哪些患者更容易发生HSCT后TMA的可能性,并确定了哪些危险因素更常与TMA后的不良结局相关。

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