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Related Experiment Videos

Cellular basis of hyper IgM immunodeficiency.

D Pascual-Salcedo, E G de la Concha, M C Garcia-Rodriguez

    Journal of Clinical & Laboratory Immunology
    |January 1, 1983
    PubMed
    Summary
    This summary is machine-generated.

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    This study investigated primary hypogammaglobulinaemia with hyper IgM, finding a B cell maturation defect and impaired T cell help for immunoglobulin production in most patients.

    Area of Science:

    • Immunology
    • Clinical Medicine

    Background:

    • Primary hypogammaglobulinaemia with hyper IgM is a rare immunodeficiency.
    • Patients exhibit low levels of IgG and IgA, with normal or elevated IgM.
    • The underlying cellular defects are not fully understood.

    Purpose of the Study:

    • To investigate the B cell and T cell defects in patients with primary hypogammaglobulinaemia and hyper IgM.
    • To elucidate the mechanisms behind impaired immunoglobulin production.

    Main Methods:

    • Studied six patients with primary hypogammaglobulinaemia and hyper IgM.
    • Assessed in vitro pokeweed-mitogen (PWM)-induced immunoglobulin production by peripheral blood lymphocytes.
    • Performed co-culture experiments with patient B cells and normal T cells, and vice versa.

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  • Analyzed B lymphocyte surface immunoglobulin class expression.

    • Main Results:

    • Patients showed very low serum IgG and IgA concentrations.
    • In vitro immunoglobulin production, including IgM, by lymphocytes was significantly reduced.
    • Even with normal T cells, patient B cells failed to produce adequate IgM.
    • T cells from most patients provided insufficient help for immunoglobulin production by both patient and normal B cells.
    • No suppressive effect of patient T cells on normal B and T cell co-cultures was observed.

    Conclusions:

    • A B cell maturation arrest is implicated in primary hypogammaglobulinaemia with hyper IgM.
    • A defect in T cell-mediated help for immunoglobulin production (IgM, IgG, IgA) is present in most patients.
    • The condition results from combined B cell and T cell abnormalities.