Disorders of regulatory T cell function in patients with the Wiskott-Aldrich syndrome.
Three patients with the Wiskott-Aldrich syndrome were studied. One of them had no past history of relevant infections. The other two presented different degrees of humoral and cellular immunodeficiency and their T cells in vitro showed a defect in regulatory activity of Ig production in PWM stimulated cultures. This defect was not observed in the third patient. All three had normal numbers of B cells, producing normal amounts of Ig in vitro when co-cultured with normal T cells. It is suggested that the immunoregulatory T cell abnormality might play an important role in the pathogenesis of the humoral immunodeficiency.
D001402:B-Lymphocytes / Q000276:immunology; D002478:Cells, Cultured; D002675:Child, Preschool; D006801:Humans; D007111:Immunity, Cellular; D007073:Immunoglobulin E / Q000096:biosynthesis; D007075:Immunoglobulin M / Q000172:deficiency; D007136:Immunoglobulins / Q000096:biosynthesis; D007153:Immunologic Deficiency Syndromes / Q000276:immunology; D007223:Infant; D007958:Leukocyte Count; D008213:Lymphocyte Activation; D008297:Male; D011043:Pokeweed Mitogens; D013601:T-Lymphocytes / Q000276:immunology*; D014923:Wiskott-Aldrich Syndrome / Q000276:immunology*
D007075:Immunoglobulin M; D007136:Immunoglobulins; D011043:Pokeweed Mitogens; D007073:Immunoglobulin E