Combined immune deficiency syndromes with primary T-cell defect and partial B-cell reactive hyperactivity. Immunological and morphological analysis of two unusual cases.
Two cases of combined immunodeficiency with lymphopenia, thymic dysplasia, and defective immunoglobulin production are reported. Both show selective hypo-gammaglobulinemia (IgG and IgA respectively) and selective hyper-gammaglobulinemia (both IgE, IgA, and IgM respectively). The cases are classified, by correlation of clinical and histopathological data as a variant of Fireman's disease.
D001402:B-Lymphocytes; D004062:DiGeorge Syndrome / Q000150:complications; D004406:Dysgammaglobulinemia / Q000150:complications; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications; D007070:Immunoglobulin A; D007073:Immunoglobulin E; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007153:Immunologic Deficiency Syndromes / Q000473:pathology*; D007223:Infant; D008231:Lymphopenia / Q000150:complications; D008297:Male; D013601:T-Lymphocytes
D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007073:Immunoglobulin E