title: Characterization of common variable immunodeficiency: identification of a subset of patients with distinctive immunophenotypic and clinical features.
abstract: The peripheral blood lymphocyte surface markers and clinical features of 38 patients with common variable immunodeficiency (CVID) were assessed. These studies identified a subset of CVID consisting of 14 of the 38 patients with a distinctive T-cell immunophenotype and clinical findings. The phenotypic changes were characterized by an abnormally low CD4/CD8 ratio (less than or equal to 0.9) due primarily to a significant increase in CD8 T cells. In addition, there was an expansion in CD8 T cells coexpressing CD57 and increased expression of the activation markers HLA-DR and interleukin-2 receptor (IL-2R) by these cells. This group of immunophenotypically abnormal CVID patients also had characteristic clinical features, including splenomegaly (P less than .02) and in vivo T-cell dysfunction based on the evaluation of delayed-type hypersensitivity (P less than .05). Approximately 71% of these patients had splenomegaly and 42% were anergic in contrast to the remaining group of CVID patients, in which 29% had splenomegaly and 7% were anergic. These findings define a subgroup of CVID patients that have specific immunophenotypic features and functional T-cell abnormalities.
authors: Metabolism Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892.
authors: J J
authors: JJ
authors: Wright
authors: D K
authors: DK
authors: Wagner
authors: R M
authors: RM
authors: Blaese
authors: C
authors: C
authors: Hagengruber
authors: T A
authors: TA
authors: Waldmann
authors: T A
authors: TA
authors: Fleisher
mesh_terms: D000328:Adult; D000368:Aged; D000911:Antibodies, Monoclonal / Q000276:immunology; D000945:Antigens, Differentiation, T-Lymphocyte / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure; D015704:CD4 Antigens / Q000276:immunology; D016827:CD8 Antigens; D005260:Female; D006684:HLA-DR Antigens / Q000276:immunology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000276:immunology / Q000473:pathology*; D016130:Immunophenotyping; D016131:Lymphocyte Subsets / Q000276:immunology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D015375:Receptors, Interleukin-2 / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology / Q000648:ultrastructure; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology / Q000473:pathology; D050378:T-Lymphocytes, Regulatory / Q000276:immunology / Q000473:pathology
chemical_list: D000911:Antibodies, Monoclonal; D000945:Antigens, Differentiation, T-Lymphocyte; D015704:CD4 Antigens; D016827:CD8 Antigens; D006684:HLA-DR Antigens; D015375:Receptors, Interleukin-2