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来自一家儿科移植中心的经验,该中心确定了一些与异基因造血干细胞移植后儿童肝静脉闭塞病发生相关的保护因素和风险因素。

Experience from a single paediatric transplant centre with identification of some protective and risk factors concerning the development of hepatic veno-occlusive disease in children after allogeneic hematopoietic stem cell transplant.

作者信息

Maximova N, Ferrara G, Minute M, Pizzol A, Kiren V, Montico M, Gregori M, Tamaro P

机构信息

Institute for Maternal and Child Health IRCCS "Burlo Garofolo", Via dell'Istria 65/1, 34137, Trieste, Italy,

出版信息

Int J Hematol. 2014 Jun;99(6):766-72. doi: 10.1007/s12185-014-1578-y. Epub 2014 Apr 9.

Abstract

Hepatic veno-occlusive disease (VOD) is a frequent and severe complication of hematopoietic stem cell transplantation (HSCT) affecting 9.6-17.3 % of cases. 200 HSCT, performed between January 1995 and March 2013 in our Paediatric HSCT Centre in Trieste, were retrospectively analysed to evaluate the frequency of VOD and to identify the associated risk factors. The frequency of VOD according to the Seattle criteria was 17 %, within the range reported in literature. The mortality rate was 37.5 % (75 out of 200 transplantations) in the general population and 73.5 % (25 out of 34) in VOD patients (p < 0.05). Veno-occlusive disease significantly decreased from 38 % (1995-2000) to 8 % (2007-2013) p < 0.05. Univariate and multivariate analyses identified sepsis and pre-transplant ferritin levels above 1000 ng/ml as two significant risk factors for VOD, while the use of tacrolimus appeared to be associated with a lower VOD risk. Veno-occlusive disease still remains an important cause of transplant-related mortality even if it appears to have decreased over the last few years.

摘要

肝静脉闭塞病(VOD)是造血干细胞移植(HSCT)常见且严重的并发症,在9.6%-17.3%的病例中出现。对1995年1月至2013年3月期间在的里雅斯特我们的儿科HSCT中心进行的200例HSCT进行回顾性分析,以评估VOD的发生率并确定相关危险因素。根据西雅图标准,VOD的发生率为17%,在文献报道的范围内。总体人群的死亡率为37.5%(200例移植中有75例),VOD患者的死亡率为73.5%(34例中有25例)(p<0.05)。VOD从1995-2000年的38%显著下降至2007-2013年的8%(p<0.05)。单因素和多因素分析确定败血症和移植前铁蛋白水平高于1000 ng/ml是VOD的两个重要危险因素,而使用他克莫司似乎与较低的VOD风险相关。即使在过去几年中VOD似乎有所下降,但它仍然是移植相关死亡的重要原因。

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