Transplantation of hematopoietic stem cells in human severe combined immunodeficiency: longterm outcomes.
Transplantation of hematopoietic stem cells in human severe combined immunodeficiency: longterm outcomes.
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DOI：
10.1007/s12026-010-8191-9
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发表时间：
2011-04
影响因子：
4.4
通讯作者：
Buckley, Rebecca H.
中科院分区：
文献类型：
--
作者：
Buckley, Rebecca H.
严重联合免疫缺陷综合征(SCID)是一种遗传原因多样的综合征，其特征是T和B细胞功能严重缺陷，在某些类型中，还包括NK细胞和功能的缺陷。已发现13种不同基因的突变导致了这种情况，除非能够完成免疫重建，否则这种情况在生命的头两年都是致命的。自1968年首次进行骨髓移植以来的42年里，所有形式的SCID的标准治疗方法都是异基因骨髓移植。人类白细胞抗原完全相合的普通骨髓移植和去除T细胞的半相合骨髓移植在影响免疫重建方面都非常成功，特别是在出生后的前3.5个月和移植前不进行化疗的情况下。本文按分子类型总结了该院在过去28.3年间连续166例接受非条件亲属供者骨髓移植的SCID婴儿的长期结果，并回顾了其他中心发表的SCID移植的长期结果。
Severe combined immunodeficiency (SCID) is a syndrome of diverse genetic cause characterized by profound deficiencies of T and B cell function and, in some types, also of NK cells and function. Mutations in thirteen different genes have been found to cause this condition, which is uniformly fatal in the first two years of life unless immune reconstitution can be accomplished. In the 42 years since the first bone marrow transplant was given in 1968, the standard treatment for all forms of SCID has been allogeneic bone marrow transplantation. Both HLA identical unfractionated and T cell depleted HLA haploidentical bone marrow transplants have been very successful in effecting immune reconstitution, especially if performed in the first 3.5 months of life and without pre-transplant chemotherapy. This paper summarizes the longterm outcome, according to molecular type, of 166 consecutive SCID infants given non-conditioned related donor bone marrow transplants at this institution over the past 28.3 years and reviews published reports of longterm outcomes of transplants in SCID performed at other centers.
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