Immunoproliferative small intestinal disease in south India: A clinical and immunomorphological study

Immunoproliferative small intestinal disease (IPSID), a proliferative disor der affecting the intestinal immune system, has only been reported sporadic ally in India. Fifteen patients with malabsorption syndrome who were diagno sed to have IPSID were included in this study. Mucosal biopsies from all pa tients, full thickness surgical biopsies from 10 and autopsy material from four patients were examined by light microscopy and immunohistochemistry. T he patients were predominantly young (aged 16-36 years) and male (13 of 15) . Diarrhoea, weight loss, vomiting and abdominal pain were the major sympto ms. The upper small bowel was involved in all cases. Involvement of large b owel was detected antemortem in three patients, but was found in all autops ied patients. Involvement of the stomach was noted in one patient at autops y. Mesenteric lymph nodes were involved in all patients who underwent lapar otomy. The plasmacytic infiltrate mas uniformly positive for alpha-heavy ch ain, and either negative for light chain production or showed monotypic lig ht chain production. Some of the blasts were also positive for alpha-heavy chain. Three patients died before therapy could be commenced. One patient w ith stage A disease is alive and clinically free of disease at 7 years. Of the remainder, there have been four long-term survivors with chemotherapy. Immunoproliferative small intestinal disease occurs in southern India and h as characteristics similar to that in other parts of the world. Early diagn osis may improve outcome in this disease.