S2864 A Rare Presentation of Immunoproliferative Small Intestinal Disease

Abstract

INTRODUCTION: Immunoproliferative small intestinal disease (IPSID), is a form of extra-nodal marginal zone B-cell lymphoma, seen in young adults in the Mediterranean basin and Southeast Asia. It characterized by the production of monoclonal heavy chain without light chains that are deposited in the lamina propria, culminating in blunting of intestinal villi, malabsorption, and protein-losing enteropathy. It manifests as colicky abdominal pain, persistent diarrhea, malabsorption, weight loss and failure to thrive. IPSID is associated with chronic Campylobacter jejuni infection and has a range of presentation from lymphoid infiltration to malignant diffuse large-B-cell lymphoma, early stages of which are completely responsive to antibiotics. CASE DESCRIPTION/METHODS: A 50-year-old woman presented after syncope and a two-week history of persistent watery diarrhea and thirty-pound weight loss over six months (Table A). Four months prior, she was hospitalized in Nigeria for similar symptoms. She was ill-appearing and malnourished with a diffusely tender abdomen. Computed tomography revealed diffuse mesenteric lymphadenopathy. An extensive infectious (Table A) and non-infectious (Table B) was negative except for a positive Campylobacter stool antigen. Endoscopy revealed moderate gastritis and colitis, duodenal biopsy revealed blunted villi and lymphocytic infiltration (Figure A). Her diarrhea improved with antibiotics and she was discharged.One month after discharge, she presented with recurrence of symptoms. An extensive workup yielded the same results as before. Biopsy of mesenteric lymph nodes revealed reactive lymphadenitis. One year later, the patient presented to the hospital again, now with neck pain and dysphagia in addition to the prior GI symptoms. She was found to have cervical lymphadenopathy biopsy of which revealed lymphoma. She was diagnosed with IPSID Stage C initiated on chemotherapy. However, she succumbed to her disease. DISCUSSION: IPSID should be considered in all patients from endemic regions after other causes of infectious and non-infectious diarrhea and mesenteric lymphadenopathy are ruled out. Early diagnosis and treatment, at a stage responsive to antibiotics, may lead to resolution of the disease. In the case of our patient, Campylobacter stool antigen was persistently positive even after she had left an endemic region (West Africa) and received multiple courses of antibiotics, underscoring the puzzling nature of the source of her recurrent bacterial infection.Table 2.: Diarrhea and mesenteric lymphadenopathy can be caused by a multitude of pathology, which should be considered and ruled out because a diagnosis of IPSID is madeTable 1.: Initial presentation versus one month later versus one year laterFigure 1.: 4x magnification, Low power view of the small intestinal mucosa with marked villous blunting and expansion of lamina propria by small, mature lymphoid cells. Figure 2: 40x magnification, High power view of the small intestinal mucosa showing sheets of predominantly mature lymphoid cells with scattered plasma cells and eosinophils.