4 Years of Diarrhea: A Case of Immunoproliferative Small Intestinal Disease: 298

Purpose: A 50 year old Nigerian woman presented with complaint of diarrhea for 4 years. She noted a 50 pound unintentional weight loss, diffuse abdominal pain and 10 episodes of watery diarrhea per day. She denied fever, bloody stool, or tenesmus. On examination, she was ill-appearing with a diffusely tender abdomen without rebound. CT abdomen/pelvis showed mesenteric lymphadenopathy and colonoscopy revealed normal mucosa. Duodenal biopsy revealed blunted villi and lymphocytic infiltration but anti-TTG antibodies were negative. Capsule endoscopy revealed edema and hyperemia of small bowel walls. Duodenal/jejunal pathology from single balloon enteroscopy showed no organisms, diffuse lymphoplasmacytic infiltration of the lamina propria and blunted villi. Mesenteric lymph node biopsy revealed reactive lymphadenitis. Given the patient's clinical and histopathological findings in addition to her African origin, negative celiac and infectious disease panels, a diagnosis of IPSID was made. Conclusion: IPSID is a MALT lymphoma that develops in the small intestine. It is an alpha heavy chain disease associated with an infiltration of the small intestine by plasma cells that secrete immunoglobulin alpha heavy chains lacking associated light chains. It is typically found in young adults living in poor sanitary conditions of areas specifically found in the Mediterranean basin, the Middle East, the Far East and Africa. Attempts have been made to link IPSID with a bacterial cause, specifically H. pylori and C. jejuni. Although in the early stages, some cases respond to antibiotics, many will not and in some instances, may progress to B cell lymphoma.Figure: [298] Paucity of jejuanal folds.Figure: [298] Jejunum with blunted villi and infiltration of the lamina propria by lymphocytes and plasma cells. Immunohistochemstry with CD3 and CD20 positive cells.