title: Hyper-IgM immunodeficiency syndrome: influence of lymphokines on in vitro maturation of peripheral B cells.
abstract: Peripheral B cells from six patients affected with the hyper-IgM immunodeficiency syndrome, characterized by an absence of IgG and IgA in serum with a concomitant elevated level of IgM, were analyzed for phenotypic and functional characteristics. We report that although the membrane antigenic pattern expression was characteristic of mature B cells, B cells from most patients exhibited an impairment in their in vitro response to several lymphokines, such as recombinant interleukin 2 (rIL-2) and low molecular weight B-cell growth factor (BCGF), that induce proliferation of anti-mu-activated B cells. This impairment was also found in response to a lymphokine mixture from a CD2-activated T-cell clone. The decrease in lymphokine-induced B-cell proliferation was accompanied by a low B-cell differentiation, whether patients' B cells were stimulated by the T-cell clone supernatant or rIL-2 and rIL6, lymphokines able to support differentiation of Staphylococcus aureus Cowan I (SAC)-activated B cells. In addition, none of the lymphokines tested were able to induce patients' B cells to switch from IgM-secreting cells to IgG- and IgA-secreting cells. We conclude that this syndrome is associated with a defect in lymphokine-dependent maturation of B lymphocytes, although the T- or the B-cell origin of the defect still cannot be determined.
authors.affiliation: Unité d'Immunologie et de Rhumatologie Pédiatrique, INSERUM U 132, Hôpital Necker-Enfants-Malades, Paris, France.
authors.forename: M L
authors.initials: ML
authors.lastname: Gougeon
authors.forename: L
authors.initials: L
authors.lastname: Morelet
authors.forename: M
authors.initials: M
authors.lastname: Doussau
authors.forename: J
authors.initials: J
authors.lastname: Theze
authors.forename: C
authors.initials: C
authors.lastname: Griscelli
authors.forename: A
authors.initials: A
authors.lastname: Fischer
mesh_terms: D000921:Antibody-Producing Cells / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology*; D002648:Child; D002675:Child, Preschool; D004406:Dysgammaglobulinemia / Q000276:immunology*; D006801:Humans; D006942:Hypergammaglobulinemia / Q000276:immunology*; D007132:Immunoglobulin Isotypes / Q000276:immunology; D007075:Immunoglobulin M / Q000172:deficiency*; D016130:Immunophenotyping; D007376:Interleukin-2 / Q000494:pharmacology; D015850:Interleukin-6 / Q000494:pharmacology; D008213:Lymphocyte Activation / Q000276:immunology; D008222:Lymphokines / Q000494:pharmacology*; D008297:Male; D011994:Recombinant Proteins / Q000494:pharmacology
chemical_list: D007132:Immunoglobulin Isotypes; D007075:Immunoglobulin M; D007376:Interleukin-2; D015850:Interleukin-6; D008222:Lymphokines; D011994:Recombinant Proteins