title: Interferon production in primary immunodeficiencies.
abstract: Alpha- and gamma-interferon (IFN) production by peripheral blood mononuclear cells (PBMC) from 18 patients affected by primary immunodeficiency syndromes was examined and compared with that of 20 normal donors. Patients included 8 with common variable immunodeficiency (CVI), 2 with congenital agammaglobulinemia, 4 with ataxia-telangiectasia, 2 with hyper-IgE syndrome, 1 with chronic EBV infection, 1 with combined immunodeficiency, and 1 with immunodeficiency with hyper-IgM. No spontaneous IFN production was observed in either patients and controls. Newcastle disease virus-induced alpha-IFN production was found to be normal in all patients. Gamma-IFN was induced by both galactose oxidase and staphylococcal enterotoxin (B). Gamma-interferon production was low or undetectable in patients with ataxia-telangiectasia, in immunodeficiency with hyper-IgM, and in hyper-IgE syndrome. No major defect of gamma-IFN was found in other types of immunodeficiency, despite the presence of occasional low producers (1 of 8 CVI patients and 1 case of congenital agammaglobulinemia). No correlation was found between IFN production and natural killer activity in individual patients. The analysis of lymphocyte subsets by monoclonal antibodies revealed gross imbalances of helper/inducer and suppressor/cytotoxic subpopulations, but no overall correlation could be established with gamma-IFN production. The observation of major defects in gamma-IFN yield only in diseases with depression of T cell-mediated immunity might contribute to a better understanding of the pathogenetical mechanisms in these diseases. Moreover, future studies should monitor these in vitro functions and their modifications by in vitro or in vivo manipulations.
mesh_terms: D000293:Adolescent; D000328:Adult; D000361:Agammaglobulinemia / Q000276:immunology; D001260:Ataxia Telangiectasia / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D005260:Female; D006566:Herpesviridae Infections / Q000276:immunology; D004854:Herpesvirus 4, Human; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology; D007073:Immunoglobulin E; D007075:Immunoglobulin M; D007153:Immunologic Deficiency Syndromes / Q000276:immunology*; D066298:In Vitro Techniques; D007370:Interferon Type I / Q000096:biosynthesis*; D007371:Interferon-gamma / Q000096:biosynthesis*; D007962:Leukocytes / Q000276:immunology; D008297:Male; D008875:Middle Aged
chemical_list: D007075:Immunoglobulin M; D007370:Interferon Type I; D007073:Immunoglobulin E; D007371:Interferon-gamma