Severe Diarrhea Controlled by Gamma Globulin in a Patient with Agammaglobulinemia, Amyloidosis, and Thymoma

Abstract

This report describes an elderly patient who developed acquired agammaglobulinemia with recurrent respiratory infections and severe diarrhea. The diarrhea, which was not associated with malabsorptlon, responded dramatically and repeatedly to gamma globulin therapy. At autopsy amyloid was found in the mucosa and blood vessels of the small intestine, as well as in the spleen, adrenals and thyroid. No amyloid was detected on biopsy of the duodenum a yr. before his death. The association of agammaglobulinemia and diarrhea, reported in 38 patients, was reviewed. The response of the diarrhea in some of these patients to antibiotic or gamma globulin therapy suggests that intestinal bacteria was responsible for the diarrhea. It seems probable that impaired defenses against bacterial infection in agammaglobulinemia cause diarrhea by permitting overgrowth or invasion of the intestinal wall by normally nonpathogenic bacterial flora. The significance and the mechanism of the intestinal amyloid deposition in this patient are unknown. The association of agammaglobulinemia and thvmoma, which was reported only with acquired agammaglobulinemia, is accompanied by splenomegaly, leukopenia and anemia. Although the thymoma often preceded the recurrent infections by some years, thymectomy did not alter the disease.