Cellular basis of hyper IgM immunodeficiency.
Six patients with primary hypogammaglobulinaemia and hyper IgM were studied. All showed very low serum IgG and IgA concentrations. The in vitro pokeweed-mitogen (PWM)-induced immunoglobulin (Ig) production, including IgM, by their peripheral blood lymphocytes was low. Even when patients' B cells were cocultured with normal T cells, IgM production did not reach normal levels. These results and studies of Ig class on the surface of B lymphocytes point to a maturation arrest of these cells. T cells from all but one patient helped very little Ig production by patients' or normal B cells. Similar numbers of these T cells did not suppress Ig production by normal T plus B cells. Therefore a defect in T cell help for IgM, IgG and IgA was seen in most patients, in addition to the B cell abnormality.
D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D002648:Child; D002675:Child, Preschool; D004406:Dysgammaglobulinemia / Q000150:complications* / Q000276:immunology; D005260:Female; D006801:Humans; D006942:Hypergammaglobulinemia / Q000150:complications* / Q000276:immunology; D007075:Immunoglobulin M; D007136:Immunoglobulins / Q000096:biosynthesis; D008297:Male; D008934:Mitogens / Q000494:pharmacology; D014066:Palatine Tonsil / Q000276:immunology; D006377:T-Lymphocytes, Helper-Inducer / Q000276:immunology
D007075:Immunoglobulin M; D007136:Immunoglobulins; D008934:Mitogens