AUTOIMMUNE HEMOLYTIC-ANEMIA IN A CHILD WITH MHC CLASS-II DEFICIENCY

A 3 year old Turkish girl is described who was suffering from major hi stocompatibility complex (MHC) class II deficiency syndrome, which is characterised by the lack of expression of HLA class II antigens on mo nonuclear cells. The presence of HLA class II genes was demonstrable a t the DNA level. Combined immunodeficiency was indicated by hypogammag lobulinaemia and the absence of delayed type hypersensitivity on skin testing. Further, she was unable to produce specific antibodies toward s foreign antigens and suffered from recurrent pulmonary, gastrointest inal, and septic infections from the third month of life. The clinical course was complicated by a Coombs test positive haemolytic anaemia d ue to the production of autoantibodies against the rhesus 'e' antigen, a non-glycosylated protein antigen. Haemolysis could be controlled by oral steroid treatment. This case is of interest as it shows that des pite the absence of HLA class II antigens and combined immunodeficienc y autoimmune reactions with production of specific autoantibodies dire cted to protein antigens are possible.