共济失调毛细血管扩张症患者及其家庭成员的自体混合淋巴细胞反应受损。
Impaired autologous mixed lymphocyte reaction (AMLR) in patients with ataxia-telangiectasia and their family members.
作者信息
Lahat N, Zelnik N, Froom P, Kinarty A, Etzioni A
机构信息
Department of Pediatrics, Carmel Hospital, Haifa, Israel.
出版信息
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。
Zotero 插件