Haematologica. 2013 Nov;98(11):1804-9. doi: 10.3324/haematol.2013.091074. Epub 2013 Jul 26.
Aplastic anemia is usually treated with immunosuppression or allogeneic transplant, depending on patient and disease characteristics. Syngeneic transplant offers a rare treatment opportunity with minimal transplant-related mortality, and offers an insight into disease mechanisms. We present here a retrospective analysis of all syngeneic transplants for aplastic anemia reported to the European Group for Blood and Marrow Transplantation. Between 1976 and 2009, 88 patients received 113 transplants. Most transplants (n=85) were preceded by a conditioning regimen, 22 of these including anti-thymocyte globulin. About half of transplants with data available (39 of 86) were followed by posttransplant immunosuppression. Graft source was bone marrow in the majority of cases (n=77). Transplant practice changed over time with more transplants with conditioning and anti-thymocyte globulin as well as peripheral blood stem cells performed in later years. Ten year overall survival was 93% with 5 transplant-related deaths. Graft failure occurred in 32% of transplants. Risk of graft failure was significantly increased in transplants without conditioning, and with bone marrow as graft source. Lack of posttransplant immunosuppression also showed a trend towards increased risk of graft failure, while anti-thymocyte globulin did not have an influence. In summary, syngeneic transplant is associated with a significant risk of graft failure when no conditioning is given, but has an excellent long-term outcome. Furthermore, our comparatively large series enables us to recommend the use of pre-transplant conditioning rather than not and possibly to prefer peripheral blood as a stem cell source.
再生障碍性贫血通常采用免疫抑制或同种异体移植治疗,具体取决于患者和疾病特点。同基因移植提供了一种罕见的治疗机会,移植相关死亡率低,并深入了解疾病机制。我们在此回顾性分析了向欧洲血液和骨髓移植协会报告的所有用于再生障碍性贫血的同基因移植。1976 年至 2009 年间,88 例患者接受了 113 次移植。大多数移植(n=85)都进行了预处理,其中 22 例包括抗胸腺细胞球蛋白。大约一半有数据的移植(86 例中的 39 例)在移植后进行了免疫抑制治疗。大多数移植使用骨髓作为供体(n=77)。随着时间的推移,移植实践发生了变化,更多的移植采用了预处理和抗胸腺细胞球蛋白,以及外周血干细胞。10 年总生存率为 93%,有 5 例与移植相关的死亡。移植失败发生率为 32%。未进行预处理以及使用骨髓作为供体的移植,其移植失败风险显著增加。未进行移植后免疫抑制治疗也显示出增加移植失败风险的趋势,而抗胸腺细胞球蛋白没有影响。总之,同基因移植如果不进行预处理,移植失败的风险很大,但长期预后良好。此外,我们的系列比较大,因此我们可以推荐使用移植前预处理,而不是不进行预处理,也可以优先选择外周血作为干细胞来源。