Goldman A S, Palkowetz K H, Rudloff H E, Brooks E G, Schmalstieg F C
Department of Pediatrics, University of Texas Medical Branch, Galveston 77555.
Eur J Immunol. 1992 Apr;22(4):1103-6. doi: 10.1002/eji.1830220435.
The repertoire of V regions of alpha/beta+ T cell receptor (TcR) on CD4+ and CD8+ T cells from the peripheral blood of six patients with a novel X-linked combined immunodeficiency was investigated by flow cytometry. The relative frequencies of V region subfamilies V alpha 2a on CD4+ T lymphocytes and V beta 5b and V beta 12a on CD8+ T lymphocytes from the peripheral blood from the affected males were decreased significantly. Also, the relative frequencies of certain other V region subfamilies on CD4+ or CD8+ T cells from the peripheral blood of some patients were either considerably below or above the ranges found in normal subjects. Although there may be other explanations including an extrathymic event, we suggest that the abnormalities in the TcR repertoire of peripheral blood T cells are consistent with a dysregulation of the intrathymic maturation/selection of T cells that leads to deficiencies in T cell populations in the peripheral blood of males with this disease.
通过流式细胞术研究了6例患有新型X连锁联合免疫缺陷病患者外周血中CD4 +和CD8 + T细胞上α/β + T细胞受体（TcR）的V区 repertoire。患病男性外周血中CD4 + T淋巴细胞上Vα2a以及CD8 + T淋巴细胞上Vβ5b和Vβ12a的V区亚家族相对频率显著降低。此外，一些患者外周血中CD4 +或CD8 + T细胞上某些其他V区亚家族的相对频率要么明显低于要么高于正常受试者的范围。尽管可能有其他解释，包括胸腺外事件，但我们认为外周血T细胞TcR repertoire的异常与胸腺内T细胞成熟/选择失调一致，这导致患有这种疾病的男性外周血中T细胞群体存在缺陷。
