title: Epstein-Barr virus infection and oncogenesis in primary immunodeficiency.
abstract: Lymphomas occurred in 3 of 16 Japanese patients with ataxia telangiectasia (AT) and Wiskott-Aldrich syndrome (WAS). The patients had a persistently reactivated Epstein-Barr virus (EBV) infection with a remarkable decrease in virus-specific cellular immunity. In these patients, the B lymphocytes were more sensitive to EBV-induced events and to cellular proto-oncogene activation than seen in the healthy counterparts. This immunologic and genetic background was considered to explain the massive lymphoproliferation in these primary immunodeficiency disorders.
mesh_terms: D000293:Adolescent; D000782:Aneuploidy; D000818:Animals; D001260:Ataxia Telangiectasia / Q000150:complications* / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D002472:Cell Transformation, Viral; D002648:Child; D002675:Child, Preschool; D005260:Female; D005786:Gene Expression Regulation; D004854:Herpesvirus 4, Human / Q000502:physiology; D006801:Humans; D007111:Immunity, Cellular; D008223:Lymphoma / Q000209:etiology* / Q000235:genetics; D008297:Male; D051379:Mice; D008819:Mice, Nude; D009374:Neoplasms, Experimental / Q000235:genetics; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins / Q000096:biosynthesis; D011817:Rabbits; D014412:Tumor Virus Infections / Q000150:complications* / Q000276:immunology; D014923:Wiskott-Aldrich Syndrome / Q000150:complications* / Q000276:immunology
chemical_list: C000715470:MAS1 protein, human; D000090063:Proto-Oncogene Mas; D011518:Proto-Oncogene Proteins