Immunoproliferative small-intestinal disease: clinical features and outcome in 30 cases

Experience with 30 patients with immunoproliferative small intestinal disease followed prospectively between 1971 and 1986 is described. All presented with malabsorption or growth retardation and had similar clinical, biochemical, and radiological features, irrespective of the presence of lymphoma or immunological abnormality. Alpha-chain disease protein was detected in 4 of the 11 patients who had a non-lymphomatous, predominantly plasmacytic infiltration of the small bowel; and in 5 of the 19 cases with diffuse intestinal lymphoma. The importance of exploratory laparotomy to include full-thickness intestinal biopsy in patients who have a benign infiltrate on peroral biopsy is demonstrated by the finding of lymphoma in operative specimens in 9 of 15 patients with mature, lymphoplasmacytic cells, and 5 of 8 patients with atypical, lymphoplasmacytic cells. The majority of patients with fully established benign disease, even those elaborating alpha-chain disease protein, appeared to have a good prognosis. No patient with immunoproliferative small intestinal disease developed immunologically demonstrated alpha-chain disease or frank lymphoma, when this was not found initially at explorative laparotomy.