Hypogammaglobulinemia With Nodular Lymphoid Hyperplasia of the Small Bowel

Abstract

Gastrointestinal symptoms occur frequently in patients with hypogammaglobulinemia. Rosen and Janeway reported that more than half of the patients with acquired hypogammaglobulinemia had diarrhea or steatorrhea.1In contrast, these symptoms occurred rarely in patients with the congenital form of hypogammaglobulinemia. The histology of the small intestine usually has been normal although a spruelike pattern has been noted occasionally. Recently, a syndrome of chronic diarrhea and dysgammaglobulinemia associated with numerous hyperplastic lymphoid nodules in the submucosa of the small bowel has been described.2 We have recently had an opportunity to study an additional example of this disorder and to characterize further the nature of the immunological defect. In the present case, the principal gastrointestinal symptom was bleeding and there was no evidence of malabsorption. The patient had severe hypogammaglobulinemia involving all three major immunoglobulins, without any demonstrable defect in the processes of cellular immunity. Patient Summary A 26-year-old white