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.