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[Studies on Epstein-Barr virus (EBV) infection and reactivity of peripheral B lymphocytes to EBV in patients with ataxia telangiectasia].

Ataxia telangiectasia (AT) is a primary immunodeficiency syndrome characterized by oculocutaneous telangiectasia, ataxia, recurrent infection and development of malignancies. Epstein-Barr virus (EBV) is a B-cell lymphocytotropic virus which causes infectious mononucleosis and is also highly associated with Burkitt's lymphoma, nasopharyngeal carcinoma and lymphoproliferative disorders in immunodeficient patients. 10 Japanese patients with AT were studied concerning the status of EBV infection by specific EBV serology, and reactivity of peripheral lymphocytes to EBV. All the AT patients had high EBV antibody titers of IgG to viral capsid antigen (VCA) and early antigen (EA), while low titers of IgG to EBV-associated nuclear antigen (EBNA), compared with age and sex matched healthy controls. However, significant differences were not apparent with antibodies to several other viruses between the AT patients and controls. These antibody characteristics were thought to be that an activated EBV infection occurred in AT patients. Then the lymphocytes were exposed to B95-8 strain EBV. There was no significant differences in EBNA induction frequency at 24 hours prior to cellular DNA synthesis, between the AT and controls. EBV-specific T cell killer function was very low as judged with the days of establishment of lymphoblastoid cells expressing EBNA on all cells after EBV exposure, when compared with the lymphocytes from controls. These AT lymphoblastoid cells easily expressed EA and VCA by cultivation at lower temperature of 33 degrees C, 12-0-tetradecanoyl-phorbol-13-acetate treatment, 60Co irradiation and by P3HR-1 strain EBV infection. Malignant transformation with high colony forming efficiency in soft agarose and tumor formation in nude mice easily occurred with some of AT lymphoblastoid cells.(ABSTRACT TRUNCATED AT 250 WORDS)