Tsuge I, Matsuoka H, Torii S, Okada J, Mizuno T, Matsuoka M, Kodera Y, Takahashi T
J Clin Lab Immunol. 1987 May;23(1):7-13.
Peripheral blood mononuclear cells (PBMs) from 6 patients with ataxia-telangiectasia (AT) were studied by 5 kinds of cell-mediated cytotoxicity systems. Decrease in cell mediated lympholysis (CML) activity to allogeneic lymphocytes was observed in all 6 AT patients who had low numbers of OKT-3+ cells. These patients also showed decreased proliferative responses to phytohemagglutinin stimulation and allogeneic lymphocytes. In contrast, antibody-dependent cell-mediated cytotoxicity (ADCC) activity and natural killer (NK) activity were comparable with those in normal controls. In addition, PBMs from these AT patients activated by in vitro stimulation with allogeneic PBMs or interleukin-2 were able to acquire lytic activity against NK-insensitive target cells. The phenotypes of these effectors determined by complement-mediated lysis were OKT-3- and Leu-11+, suggesting that they were derived from NK cell lineage. Thus, AT patients with severe T cell defects were found to maintain a normal range of NK, ADCC, MLC-activated and lymphokine-activated killer activity.
采用5种细胞介导的细胞毒性系统对6例共济失调毛细血管扩张症（AT）患者的外周血单个核细胞（PBMs）进行了研究。在所有6例OKT-3+细胞数量较少的AT患者中，均观察到对同种异体淋巴细胞的细胞介导淋巴细胞溶解（CML）活性降低。这些患者对植物血凝素刺激和同种异体淋巴细胞的增殖反应也降低。相比之下，抗体依赖性细胞介导的细胞毒性（ADCC）活性和自然杀伤（NK）活性与正常对照相当。此外，这些AT患者的PBMs经同种异体PBMs或白细胞介素-2体外刺激激活后，能够获得针对NK不敏感靶细胞的溶解活性。通过补体介导的溶解确定的这些效应细胞的表型为OKT-3-和Leu-11+，表明它们来源于NK细胞系。因此，发现具有严重T细胞缺陷的AT患者的NK、ADCC、混合淋巴细胞培养激活和淋巴因子激活杀伤活性维持在正常范围内。
