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P304 Extranodal primary intestinal lymphoma in inflammatory bowel disease: An ECCO CONFER multi-centre case series

F. Phillips1, B. Verstockt2,3, D. Ribaldone4, I. Guerra5, N. Teich6, K. Katsanos7, R. Filip8, T. Molner9, K. Karmiris10, ECCO CONFER Investigators

1NIHR Nottingham Digestive Diseases Biomedical Research Centre, Gastroenterology, Nottingham, UK, 2University Hospitals Leuven, Gastroenterology and Hepatology, Leuven, Belgium, 3KU Leuven, Chronic Diseases, Metabolism and Ageing, TARGID-IBD Unit, Leuven, Belgium, 4University of Turin, Department of Medical Sciences, Turin, Italy, 5Hospital Universitario de Fuenlabrada and Instituto de Investigacion de La Paz IdiPaz, Gastroenterology, Madrid, Spain, 6Internistische Gemeinschaftspraxis, Gastroenterology, Leipzig, Germany, 7University of Ioannina School of Health Sciences, Division of Gastroenterology, Department of Internal Medicine- Faculty of Medicine, Ioannina, Greece, 8University of Rzeszow, Department of Gastroenterology with IBD Unit of Clinical Hospital 2, Rzeszow, Poland, 9University of Szeged, First Department of Medicine, Szeged, Hungary, 10Venizeleio General Hospital, Gastroenterology, Heraklion, Greece

Background

There is a small but measurable increased risk of lymphoma in inflammatory bowel disease (IBD). Evidence coming from case reports and small case series of primary intestinal lymphoma in IBD suggest an association with inflamed tissue and immunosuppressant use, mainly thiopurines.

Methods

This was a multicentre case series supported by the European Crohn’s and Colitis Organisation (ECCO) and performed as part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. A call was made to report on cases of intestinal lymphoma in IBD and clinical data were recorded in a standardised case report form.

Results

Fifteen patients with intestinal lymphoma from 8 centres were included [12 males and 3 females, mean age 47.8 ( ± 16.4 SD, range 26–76) years at lymphoma diagnosis]. Eleven patients had Crohn’s disease: 7 had diffuse large B-cell lymphoma (DLBCL), 2 had Hodgkin’s disease and 2 had MALT lymphoma. Four patients had ulcerative colitis: DLBCL, MALT, indolent T-cell and immunoblastic lymphoma with plasma cell differentiation were diagnosed as a single case in each. Nine patients presented with symptoms of bowel obstruction, two presented with a non-obstructing mass, and there were single cases of isolated weight loss, symptoms resembling disease flare, indolent jaundice (from a duodenal lymphoma) and an incidental finding. The location of lymphoma was rectum in 3 cases, colon in 3 cases, small intestine in 4 cases, ileocolonic anastomosis in one case, stomach in one case and in multiple upper and lower GI sites in 3 cases. Lymphoma was located within the area affected from IBD in 10 patients, with four of these having active intestinal inflammation at the time of diagnosis. At lymphoma diagnosis, 7 patients were on azathioprine (one of whom also had adalimumab for 3 months), one was on infliximab for 1 month, one was on low dose steroids, one was on an aminosalicylate and 5 had not received IBD-related medications. Lymphoma was diagnosed at a mean time of 10.4 ( ± 7.07 SD, 1–24) years after IBD diagnosis in 11 patients, prior to IBD in one and concurrently with IBD in 3. Remission was achieved in 11 patients after treatment, 2 died from their lymphoma despite treatment and 2 are being monitored without therapy.

Conclusion

This case series of primary intestinal lymphomas in IBD illustrates a strong male predilection and a wide histological type range, with the majority being DLBCL. More than half of the cases cannot be attributed to IBD-related treatment whilst in a third of patients, lymphoma developed at a distant site from the area affected from IBD, thereby questioning the role of active inflammation or medication use in its development. The majority of cases successfully recovered after appropriate treatment.