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儿童获得性重型再生障碍性贫血的异基因骨髓移植

Allogeneic bone marrow transplantation (BMT) for acquired severe aplastic anaemia (SAA) in children.

作者信息

Locasciulli A, Vossen J, Bacigalupo A, Hows J, VanLint M T, Gluckman E, Nissen C, McCann S, de Planque M, van'tVeer L

出版信息

Bone Marrow Transplant. 1989 Dec;4 Suppl 4:123-5.

PMID:2697423
Abstract

The SAA Registry of the EBMT now contains data on 171 children younger than 15 years of age with acquired SAA and undergoing BMT between 1970 and 1988. The overall actuarial survival is 63% at 10 years. In a multivariate Cox analysis, the year of transplant was the most important prognostic factor with a significant advantage for children grafted in 1984-88 (81%) vs 1981-83 (67%) and 1970-80 (41%) (p = 0.02). Cyclosporine A given for GVHD prophylaxis, no treatment before transplant and an interval less than 90 days from diagnosis to BMT were all favourable variables in univariate analysis. As regard to transplant procedures, the better results were obtained using Cyclophosphamide and Cyclosporine A (78%) followed by Cyclophosphamide plus irradiation plus Cyclosporine A (77%). Sex, etiology and the severity of the aplasia had no impact on survival in both uni and multivariate analysis.

摘要

欧洲血液与骨髓移植协会(EBMT)的严重再生障碍性贫血(SAA)登记处目前包含了1970年至1988年间171例15岁以下获得性SAA且接受骨髓移植(BMT)儿童的数据。10年的总精算生存率为63%。在多变量Cox分析中,移植年份是最重要的预后因素,1984 - 1988年接受移植的儿童(81%)相比1981 - 1983年(67%)和1970 - 1980年(41%)具有显著优势(p = 0.02)。在单变量分析中,用于预防移植物抗宿主病(GVHD)的环孢素A、移植前未进行治疗以及从诊断到BMT的间隔时间少于90天均为有利变量。关于移植程序,使用环磷酰胺和环孢素A(78%),其次是环磷酰胺加放疗加环孢素A(77%),可获得更好的结果。在单变量和多变量分析中,性别、病因以及再生障碍的严重程度对生存率均无影响。

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引用本文的文献

1
Aplastic anaemia: continued cause for concern.再生障碍性贫血:持续令人担忧的病因。
Arch Dis Child. 1990 Oct;65(10):1105-6. doi: 10.1136/adc.65.10.1105.