Chest disease in patients with agammaglobulinemia.

As a new disease entity is studied it becomes apparent that certain clinical and laboratory features are particularly helpful to the clinician in the recognition of the disorder. A consideration of the recent literature indicates that recurrent pulmonary disease has assumed this role in the disease syndrome associated with agammaglobulinemia. Recurrent pulmonary infections including bronchitis, pneumonitis, and pneumonia at times leading to pulmonary fibrosis, bronchiectasis, and empyema, appear to be extremely frequent manifestations of agammaglobulinemia and as reporting becomes more complete it seems likely that pulmonary disease may become the clinical sin qua non of this syndrome. Consequently, it seems especially pertinent that a complete review of reported cases of agammaglobulinemia be submitted to chest physicians who are among those most likely to encounter patients suffering from this metabolic disturbance. It is the purpose of this report to present a thorough review of the reported experience with agammaglobulinemia and to emphasize the pulmonary manifestations observed in our cases and those studied by others. Agammaglobulinemia is a metabolic disorder featured by an enhanced susceptibility to bacterial infection, absence of gamma globulin from the serum, absence of antibodies from the blood and tissues, and failure of immunologic response to antigenic stimulation)3 Evidence has been submitted indicating that this disorder reflects a disturbance in function of the hematopoietic reticulum (mesenchyme) which is expressed in each patient as a failure of plasma cell formation and consequent failure of antibody production in response to antigenic stimulation.48 On the basis of clinical and laboratory data this disorder can be subdivided into a congenital and an acquired type.3 In both diseases the chief clinical manifestation is the occurrence of recurrent severe bacterial infections. The congenital disease is, in general, expressed early in life, and, like hemophilia, appears to be an inborn error of protein synthesis transmitted as a sex-linked recessive trait.2’ #{176} Thus, this form of agammaglobulinemia occurs only in male children, frequently in more than one member of a