Lahat N, Zelnik N, Froom P, Kinarty A, Etzioni A
Department of Pediatrics, Carmel Hospital, Haifa, Israel.
Clin Exp Immunol. 1988 Oct;74(1):32-5.
We used the autologous mixed lymphocyte reaction (AMLR) to test T cell function in four patients with Ataxia-telangiectasia (AT), in 11 first-degree relatives and in 20 controls. There was a marked reduction of AMLR in the patients and in three relatives compared to the age-matched controls. In the AT patients the defect in AMLR was intrinsic to the CD4 subpopulation, since exogenous IL-2 did not improve the response of isolated CD4 cells. In contrast to normal controls, pre-incubation of autologous B cells with Epstein-Barr virus (EBV) did not enhance the reduced AMLR in the AT patients and the three first-degree relatives. We conclude that in both patients with AT and in some of their family members there is an intrinsic defect in CD4 T cells. This defect leads to diminished reactivity to EBV infected autologous B cells, and may explain in part the high incidence of malignancies observed in such families.
我们采用自体混合淋巴细胞反应（AMLR）检测了4例共济失调毛细血管扩张症（AT）患者、11名一级亲属和20名对照者的T细胞功能。与年龄匹配的对照者相比，患者及3名亲属的AMLR显著降低。在AT患者中，AMLR缺陷存在于CD4亚群，因为外源性白细胞介素-2并不能改善分离出的CD4细胞的反应。与正常对照不同，用爱泼斯坦-巴尔病毒（EBV）预孵育自体B细胞并不能增强AT患者及3名一级亲属降低的AMLR。我们得出结论，AT患者及其部分家庭成员的CD4 T细胞存在内在缺陷。这种缺陷导致对EBV感染的自体B细胞反应性降低，可能部分解释了此类家族中观察到的恶性肿瘤高发病率。
