Immunoproliferative small intestinal disease in South India: a clinical and immunomorphological study

Immunoproliferative small intestinal disease (IPSID), a proliferative disorder affecting the intestinal immune system, has only been reported sporadically in India. Fifteen patients with malabsorption syndrome who were diagnosed to have IPSID were included in this study. Mucosal biopsies from all patients, full thickness surgical biopsies from 10 and autopsy material from four patients were examined by light microscopy and immunohistochemistry. The patients were predominantly young (aged 16-36 years) and male (13 of 15). Diarrhoea, weight loss, vomiting and abdominal pain were the major symptoms. The upper small bowel was involved in all cases. Involvement of large bowel was detected antemortem in three patients, but was found in all autopsied patients. Involvement of the stomach was noted in one patient at autopsy. Mesenteric lymph nodes were involved in all patients who underwent laparotomy. The plasmacytic infiltrate was uniformly positive for alpha-heavy chain, and either negative for light chain production or showed monotypic light chain production. Some of the blasts were also positive for alpha-heavy chain. Three patients died before therapy could be commenced. One patient with 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 has characteristics similar to that in other parts of the world. Early diagnosis may improve outcome in this disease.