Seto S, Miyake T, Hirao T
Tokai J Exp Clin Med. 1985 Jun;10(2-3):233-8.
A male infant X-linked, adenosine deaminase-positive severe combined immunodeficiency underwent partial immunological reconstitution by fetal liver transplantation at twenty months of age. Reconstitution of T lymphocytes was observed from sixteen weeks after transplantation, in the increase of T lymphocytes, positive delayed-type skin reaction and in vitro responsiveness to mitogens and allogeneic cells, sequentially. Chimerism was defined by chromosomal analysis and HLA typing, in which one haplotype seemed to be shared between the donor and the host by chance. However, defective immunoglobulin production has not yet been corrected. The assay on T-B lymphocyte interaction in vitro suggested that the failure might be attributed to an intrinsic defect of B lymphocytes, which were cells of donor origin after transplantation. Three years following transplantation, the patient is free of severe infections, although he requires regular injections of gamma globulin.
一名患有X连锁腺苷脱氨酶阳性严重联合免疫缺陷的男婴在20个月大时接受了胎儿肝移植,实现了部分免疫重建。移植后16周观察到T淋巴细胞的重建,表现为T淋巴细胞数量增加、迟发型皮肤反应阳性以及对有丝分裂原和异基因细胞的体外反应性依次增强。通过染色体分析和HLA分型确定了嵌合现象,其中一个单倍型似乎偶然地在供体和宿主之间共享。然而,免疫球蛋白产生缺陷尚未得到纠正。体外T-B淋巴细胞相互作用检测表明,这种失败可能归因于B淋巴细胞的内在缺陷,而移植后B淋巴细胞是供体来源的细胞。移植三年后,尽管患者需要定期注射丙种球蛋白,但未发生严重感染。