Hypogammaglobulinemia with Thymoma, Hemolytic Anemia, and Disseminated Infection with Cytomegalovirus

Abstract

A female patient had a thymoma removed in 1956. Subsequently she developed multiple bacterial infections and in 1963 hypogammaglobulinemia was diagnosed. After repeated respiratory infections the patient died. At postmortem examination extensive infection due to cytomegalovirus was found in the lungs and adrenal glands. There was a virtual absence of plasma cells at autopsy. Laboratory investigation revealed a reduced [gamma] globulin peak on paper electrophoresis. Immunoelectrophoresis revealed a reduction of G globulin and a marked reduction of [gamma] A globulin. The patient also had a hemolytic anemia with a reticulocyte count of 30%. Despite the hypogammaglobulinemia, she was able to make abnormal antibodies demonstrated by a positive Coombs test and a positive antinuclear factor test. Most cases of cytomegalovirus infection in adults occurred in patients with debilitating illnesses such as cancer or leukemia. Recently a high incidence of such infections was reported in patients receiving immuno-suppressive therapy for renal transplantation. Cytomegaloviruses may be pathogenic agents whenever artificial or naturally acquired immunosuppression occurs.