HLA-haploidentical bone marrow transplantation for severe combined immunodeficiency using E rosette fractionation and cyclosporine.
HLA-haploidentical bone marrow transplantation for severe combined immunodeficiency using E rosette fractionation and cyclosporine.
复制标题
使用 E 玫瑰花结分离和环孢菌素进行 HLA-半相合骨髓移植治疗严重联合免疫缺陷。
作者：
A. Fischer;A. Durandy;J. D. Villartay;Etienne Vilmer;F. L. Deist;I. Gerota;C. Griscelli
八名患有严重联合免疫缺陷的患者在用神经氨酸酶处理的绵羊红细胞进行玫瑰花结以去除骨髓 T 细胞后，接受了来自其 HLA 单倍型相同父亲的骨髓细胞。由于该方法输注的T淋巴细胞比例较小（0.1%～0.3%），为预防移植物抗宿主病（GVHD）的发生，需连续静脉输注环孢素两个月。三名残留无功能 T 淋巴细胞的患者在移植前接受了白消安和环磷酰胺治疗。 7 例患者观察到移植，未见严重 GVHD。两名患者在骨髓移植后早期因先前感染而死亡，第三名患者在第 90 天死于 B 细胞淋巴增殖综合征。其他五名患者情况良好。通过重建 5/5 患者的细胞介导免疫和 4/5 患者的体液免疫，实现了稳定的植入。
Eight patients with severe combined immunodeficiency received bone marrow cells from their HLA haplotype-identical fathers after bone marrow T cell depletion by rosetting with neuraminidase-treated sheep red cells. Because the method led to the infusion of a small percentage of T lymphocytes (0.1% to 0.3%), cyclosporin was given by continuous intravenous infusion for two months in order to prevent the occurrence of graft-v-host disease (GVHD). Three patients who did have residual nonfunctional T lymphocytes received busulfan and cyclophosphamide before transplantation. Engraftment was observed in seven patients, and severe GVHD was not seen. Two patients died early after the bone marrow transplantation because of prior infections, and a third died at day 90 from a B cell lymphoproliferative syndrome. The five other patients are doing well. Stable engraftment has been achieved with reconstitution of cell-mediated immunity in 5/5 and humoral immunity in 4/5 patients.