BARE LYMPHOCYTE SYNDROME WITH LACK OF HLA CLASS-I AND CLASS-II ANTIGENS PRESENTATION OF 2 CASES

Bare lymphocyte syndrome (BLS) is a rare disorder characterized by def icient expression of human leukocyte antigens (HLA antigens) and combi ned immunodeficiency to various degrees. Recurrent severe infections e specially due to opportunistic organisms are common. Here, we present two patients with BLS who lack both class I and II antigens (Type III) . They had the typical clinical and immunologic findings of BLS. The f irst patient showed marked improvement in pulmonary symptoms resulting from cytomegalovirus infection by means of gancyclovir treatment. How ever, intramuscular injections of interferon-alpha (IFN-alpha) had no beneficial effect in either the expression of HLA antigens or the clin ical status. The second patient died of septicemia while being prepare d for bone marrow transplantation.