title: Immunoregulatory defects in a family with selective IgA deficiency.
abstract: Defects in regulation of the humoral immune system were examined in a family with selective IgA deficiency. Two patients (mother and son) were clinically well and had selective abnormalities of B lymphocytes that can differentiate into IgA plasma cells  plus specific T cell suppression of IgA production. One patient (daughter) had several clinical problems and received nine monthly transfusions of normal plasma. Prior to therapy, this patient had abnormal immunoregulation of both B and T lymphocyte population that cooperated in the formation of all cases of immunoglobulin-producing cells. After treatment, her clinical problems were resolved and her cellular abnormalities appeared identical in those in her mother and brother.
mesh_terms: D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D001803:Blood Transfusion; D002454:Cell Differentiation; D004406:Dysgammaglobulinemia / Q000235:genetics* / Q000276:immunology / Q000628:therapy; D005260:Female; D006801:Humans; D017098:IgA Deficiency; D007136:Immunoglobulins / Q000032:analysis; D007223:Infant; D008297:Male; D010949:Plasma; D010950:Plasma Cells / Q000473:pathology; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D007136:Immunoglobulins