The Congenital Agammaglobulinemias

Abstract

THE FIRST type of agammaglobulinemia reported was an X-linked recessive disease characterized by recurrent bacterial infections, very low serum immunoglobulins,* and absence of antibody responses.¹Many other types have since been recognized^2-5and a short classification of the agammaglobulinemias is listed in Table 1. Each type appears to be distinct, and each is probably the result of a different defect in the maturation and function of immunologic mechanisms. The purpose of this report is to present studies of seven patients with agammaglobulinemia. These studies indicate that cases currently called congential sporadic agammaglobulinemia may be a heterogeneous group with different defects of immunity. Agammaglobulinemia is used here in the generic sense to mean a group of related diseases which are characterized by low serum immunoglobulins, impaired antibody response, and abnormal lymphoid morphology. The use of agammaglobulinemia in this sense is comparable to the use of anemia to indicate diseases