Impaired autologous mixed lymphocyte reaction (AMLR) in patients with ataxia-telangiectasia and their family members.
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.
Lahat
N
N
Department of Pediatrics, Carmel Hospital, Haifa, Israel.
Zelnik
N
N
Froom
P
P
Kinarty
A
A
Etzioni
A
A
D001260:Ataxia Telangiectasia / Q000235:genetics / Q000276:immunology*; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006566:Herpesviridae Infections / Q000276:immunology; D004854:Herpesvirus 4, Human; D006801:Humans; D007959:Lymphocyte Culture Test, Mixed; D008297:Male; D008938:Mitosis; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology*