Hypogammaglobulinemia with nodular lymphoid hyperplasia of the intestine. Report of a case with rectosigmoid involvement.

Abstract

In a review of Mayo Clinic patients with hypogammaglobulinemia and intestinal dysfunction, Hermans et al. (4) defined a new clinical entity. The major features included: (a) unusual susceptibility to respiratory infections, (b) gastrointestinal disorders characterized by cramps, bloating, diarrhea, and occasional steatorrhea, (c) Giardia lamblia in the stools, (d) marked decrease in gamma G immunoglobulin and the absence or virtual absence of gamma A and gamma M immunoglobulins, and (e) diffuse small bowel nodules due to hyperplastic lymphoid follicles within the submucosa. The nodular lesions have been confined to the small bowel or questionably the ascending colon in previous reports (3, 5). The present study is the first reported case of this entity with rectosigmoid involvement. R. S., a 22-year-old white male, was essentially well until otitis media developed at the age of eighteen years. Shortly thereafter, while in the Army, he had five episodes of bacterial pneumonia over a five-month period. Seru...