title: [Reconstitution of immunoglobulin production in patients with variable immunodeficiency syndrome (CVI) by the CD40 system and IL-10].
abstract: Common variable immunodeficiency (CVI) is widely believed to involve a primary B cell defect, however, there is increasing evidence of T cell dysfunction in these patients. To test the hypothesis that B cells of patients with CVI are not defective, we investigated whether immunoglobulin production can be induced by activation through the CD40 system. Our results show that 6 of 7 children with CVI produced IgG when B cells were activated through CD40 plus IL-10. In most patients, IgM and IgA production were also induced. However, we could demonstrate that failure of immunoglobulin production was not due to a defective CD40 ligand expression on T cells. Taken together, our data show that B cells of patients with CVI are functionally normal when activated through CD40 plus IL-10. IL-10 seems to be more effective than IL-2 in restoration of immunoglobulin production in such patients.
authors.affiliation: Abteilung Allgemeine Pädiatrie II, Universität Frankfurt/Main.
authors.forename: S
authors.initials: S
authors.lastname: Zielen
authors.forename: P
authors.initials: P
authors.lastname: Sehrt
authors.forename: J
authors.initials: J
authors.lastname: Rahmig
authors.forename: D
authors.initials: D
authors.lastname: Hofmann
authors.forename: F
authors.initials: F
authors.lastname: Brière
authors.forename: S C
authors.initials: SC
authors.lastname: Meuer
mesh_terms: D000293:Adolescent; D000917:Antibody Formation; D015703:Antigens, CD / Q000378:metabolism / Q000502:physiology*; D000944:Antigens, Differentiation, B-Lymphocyte / Q000378:metabolism / Q000502:physiology*; D019013:CD40 Antigens; D023201:CD40 Ligand; D017074:Common Variable Immunodeficiency / Q000276:immunology / Q000628:therapy*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology; D016753:Interleukin-10 / Q000627:therapeutic use*; D008213:Lymphocyte Activation; D008562:Membrane Glycoproteins / Q000502:physiology*; D011990:Receptors, Transferrin; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D015703:Antigens, CD; D000944:Antigens, Differentiation, B-Lymphocyte; D019013:CD40 Antigens; C068126:CD71 antigen; D008562:Membrane Glycoproteins; D011990:Receptors, Transferrin; D016753:Interleukin-10; D023201:CD40 Ligand