Friedrich Wilhelm, Hönig Manfred, Müller Susanna M
Department of Pediatrics, University of Ulm, Ulm, Germany.
Immunol Res. 2007;38(1-3):165-73. doi: 10.1007/s12026-007-0030-2.
Immune reconstitution was studied in 31 long-term surviving patients after bone marrow transplantation for severe combined immunodeficiency. Donors in 7 cases were HLA-identical and in 25 cases HLA-haploidentical family members, and in 13 of these latter cases cytoreductive conditioning had been used prior to transplantation. At a mean follow-up of 15 years after transplantation (range 10 to 22 years), T cell numbers and functions had remained stable and within normal limits in the majority of patients. Marked variability however was observed with regard to reconstitution of B cell immunity. Furthermore numbers of circulating naïve CD4+ T cells were variable and markedly diminished in a substantial proportion of patients at recent evaluations. Normal B cell immunity and persistently normal naïve T cell numbers were strongly correlated with the continued detection of donor type CD34+ precursor cells in the patients marrow, which were absent in non conditioned patients. These findings indicate that stable donor precursor cell engraftment in the marrow may be of relevance for complete and stable long-term immune reconstitution in transplanted SCID patients.
对31例接受骨髓移植治疗严重联合免疫缺陷的长期存活患者的免疫重建情况进行了研究。7例供者为HLA全相合，25例为HLA单倍体相合家庭成员，其中13例在移植前使用了细胞减灭预处理。移植后平均随访15年（范围10至22年），大多数患者的T细胞数量和功能保持稳定且在正常范围内。然而，在B细胞免疫重建方面观察到明显的变异性。此外，在最近的评估中，循环中初始CD4+ T细胞数量可变，且在相当一部分患者中显著减少。正常的B细胞免疫和持续正常的初始T细胞数量与在患者骨髓中持续检测到供者型CD34+前体细胞密切相关，未进行预处理的患者中不存在这些细胞。这些发现表明，骨髓中稳定的供者前体细胞植入可能与移植的重症联合免疫缺陷患者的完全和稳定的长期免疫重建相关。
