Clinical and immunologic features of dysgammaglobulinemia type I: Report of a case diagnosed in the first year of life

A case of dysgammaglobulinemia type I (absent γ G -, decreased γ A - and increased γ M -globulin) is described. The diagnosis was established by quantitative measurement of the serum immune globulins when the patient was nine months old. The clinical features consisted of recurrent respiratory tract infections, failure to thrive and enlargement of the tonsils, liver and spleen. The immunologic defect was documented by deficient antibody response to various antigens and inability to manifest delayed hypersensitivity. Treatment with γ-globulin injections resulted in decrease in γ M levels. This “feedback inhibition” is of importance in formulating concepts regarding the control mechanism governing levels of the immune globulins in normal states.