Selective defect of precursor T cells associated with apparently normal B lymphocytes in severe combined immunodeficiency disease.
Selective defect of precursor T cells associated with apparently normal B lymphocytes in severe combined immunodeficiency disease.
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严重联合免疫缺陷病中与表面正常 B 淋巴细胞相关的前体 T 细胞的选择性缺陷。
DOI：
10.1016/s0022-3476(78)81146-9
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发表时间：
1978
期刊：
影响因子：
--
通讯作者：
F. Daguillard
中科院分区：
文献类型：
--
作者：
C. Griscelli;A. Durandy;J. Virelizier;J. Ballet;F. Daguillard
两名患者，一名患有常染色体和另一名患有性别连锁的严重联合免疫缺陷，其外周血中有超过95%的B细胞。尽管B淋巴细胞的绝对数量增加，但患者无法产生血清抗体。在每个患者中，基因或表型相同的骨髓移植后，胸腺阴影的可视化和细胞和体液功能的外观。染色体或同种异体研究表明，T细胞来源于供体，而血清免疫球蛋白是由宿主B细胞合成的。在这些患者中，发病机制似乎是骨髓前体T细胞的选择性缺陷，而没有伴随的内在B细胞缺陷。这两名患者现在分别是5岁和11个月大，没有感染，移植的成功结果表明，这类患者的首选治疗形式是骨髓干细胞移植，骨髓干细胞填充胸腺，慢慢成熟为T细胞，与宿主B细胞充分合作合成抗体。
Two patients, one with an autosomal and the other a sex-linked form of severe combined immunodeficiency, had more than 95% B cells in their peripheral blood. Despite an increased absolute number of B lymphocytes, the patients were unable to produce serum antibodies. In each patient, geno-or pheno-identical bone marrow transplantation was followed by the visualization of a thymus shadow and the appearance of both cellular and humoral functions. Chromosome or allotype studies showed that the T cell originated from the donor whereas serum immunoglobulins were synthesized by host B cells. In these patients the pathogenesis appears to be a selective defect of bone marrow precursor T cells without concomitant intrinsic B cell defect. The successful outcome of the graft in these two patients, who are now, respectively, 5 years and 11 months of age and free of infections, indicates that the preferred form of therapy in such patients is transplantation of bone marrow stem cells, which populate the thymus and mature slowly into T cells that cooperate fully with host B cells in synthesis of antibody.