[Nodular lymphoid hyperplasia of the small bowel with IgA deficiency and hemolytic anemia (author's transl)].

The syndrome of nodular lymphoid hyperplasia of the small bowel with hypogammaglobulinemia is one of the hypogammaglobulinemic enteropathies. Chronic diarrhea and malabsorption are the most characteristic features of this disease, and they are frequently associated to hypogammaglobulinemia of various types (acquired, congenital non sex-linked) and to selective IgA deficiency. The immunological deficiency gives rise to the more characteristic features of the disease, namely: a) hypogammaglobulinemia; b) respiratory infections and dental caries; c) Giardia lamblia infestation of the small bowel; d) the characteristic radiological features; and, e) the histological aspect of the intestinal mucosa with absence of plasma cells. Periodical follow-up is needed because of the increased incidence of tumors in immunological deficiency states. A new case of nodular lymphoid hyperplasia associated to hemolytic anemia and granulomatous hepatitis is reported, and its possible pathogenesis is discussed.