X-linked B lymphocyte deficiency

In two families with x-linked B lymphocyte deficiency, siblings were found to have either panhypo-γ-globulinemia or dys-γ-globulinemia. Despite differences in serum immunoglobulin patterns and severity of symptoms, a marked deficiency in surface immunoglobulin-bearing lymphocytes occurred in all affected males. These studies suggest that the primary defect in these patients is in the development of circulating B lymphocytes and that normal levels of serum IgA and IgD can be attained despite this deficiency.