P-023 B Cell Lymphoma in Colonic Stricture in a Child with Crohn's Disease

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Abstract

Background:

A male of South Indian descent with Crohn's disease first presented at 7 years of age with progressive bloody diarrhea and weight loss. His phenotype at presentation was inflammatory, with nonspecific gastroduodenitis and colonic involvement. Although he had periods of clinical remission and serological remission, there was progression to small bowel involvement with progressive dilatation and worsening activity in the descending colon. He was exposed to a number of therapies—mesalamine, methotrexate, azathioprine, infliximab, and adalimumab—in isolation as well as combined biologics and immunomodulators. At 15 years of age, he failed re-initiation of infliximab and 5 months of concomitant azathioprine. Colonoscopy prior to intended surgical intervention revealed proctosigmoiditis with a sigmoid stricture; histology showed diffuse CD20+, large B-cell lymphoma. Although Epstein-Barr virus (EBV) serology suggested an old infection, PCR serology was negative, as was in situ hybridization of the tissue. Tumor cells did not express MYC or BCL-2. Complete staging evaluation confirmed Murphy Stage III (intra-abdominal).

Background:

After multidisciplinary discussion between gastroenterologists, oncologists, and surgeon, he underwent chemotherapy following LMB/FAB regimen as per ANHL01P1. While systemic chemotherapy resulted in resolution of the focal sigmoid lymphoma, there was no change in the severity or extent of Crohn's disease. He underwent resection of persistently active ileal disease, and sigmoid stricture with a primary ileocecal resection and descending colostomy. Postoperatively, he experienced recurrence of Crohn's disease at the ileocecal anastomosis, ascending colon, and descending colon. He has been started on vedolizumab with slow progression to clinical remission.

Background:

IBD does not seem to increase the risk of lymphoma. However, there is evidence that exposure to biologic agents, thiopurines, or both are associated with an increased risk of lymphoma. The absolute risks are unknown. Further, the contribution of risk due to disease severity is unknown. Most cases appear to be EBV-driven. The relative risk due to biologic agents, thiopurines, or exposure to both is unclear. Our patient had severe disease, was treated with several years of anti-tumor necrosis factor therapy, and had a brief exposure to thiopurine combined with infliximab. However, he is unusual in that pediatric cases are rare, and both serologic PCR and histology were EBV-negative. Fortunately, the lymphoma was identified via endoscopy surveillance, as opposed to symptoms of aggressive tumor such as increased hematochezia, fever, obstruction, or perforation. In conclusion, while lymphomas are rare in pediatric IBD, and are typically associated with EBV-exposure, gastroenterologists should be aware of malignant complications in patients with phenotypically aggressive disease on combination biologic and thiopurine therapy.

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