MAJOR HISTOCOMPATIBILITY CLASS-II ANTIGEN DEFICIENCY - 9 NEW TUNISIANCASES

Background. - Bare lymphocyte syndrome is a rare inherited primary imm unodeficiency. The majority of the patients reported to date are from North Africa. We report nine new Tunisian cases. Population and method s. - Over a period of 5 years, we have established the diagnosis of ba re lymphocyte syndrome in nine patients who belong to seven different families. Class II HLA antigen expression was studied on resting perip heral mononuclear cells and PHA blasts. Results. - The clinical sympto ms started at the mean age of 4.5 months (2-10 months) with chronic di arrhea. The evolution was characterized by appearance of other recurre nt infections: pneumopathies (seven cases), thrush (seven cases), otit is (five cases) and septicemia (four cases). Allergic manifestations w ere observed in four cases. Sir patients died at the mean age of 30 mo nths from severe denutrition. Class II HLA antigens were not expressed on resting and activated lymphocytes. The absolute count of TCD4+ lym phocytes was decreased in seven patients. Lymphoproliferative response to specific antigens was absent. Four patients had panhypogammaglobul inemia. Conclusion. - This study confirms the frequency of this diseas e among the North African population. The severity of the recurrent in fection suggests the diagnosis of bare lymphocyte syndrome. This disea se is fatal in the absence of bone marrow transplantation (C) 1998 Els evier, Paris.