Case Report
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World J Gastroenterol. Jan 21, 2014; 20(3): 857-862
Published online Jan 21, 2014. doi: 10.3748/wjg.v20.i3.857
Primary effusion lymphoma-like lymphoma in a patient with inflammatory bowel disease
Elchanan Nussinson, Fahmi Shibli, Azmi Shahbari, Wasseem Rock, Mazen Elias, Irit Elmalah
Elchanan Nussinson, Fahmi Shibli, Azmi Shahbari, Gastroenterology Institute, Emek Medical Center, Afula 18101, Israel
Wasseem Rock, Mazen Elias, Department of Internal Medicine C, Emek Medical Center, Afula 18101, Israel
Irit Elmalah, Department of Pathology, Emek Medical Center, Afula 18101, Israel
Author contributions: Nussinson E, Shibli F, Shahbari A, Rock W, Elias M and Elmalah I contributed to the conception and design of the study, data acquisition, and drafting of the article; all authors approved the final version of the manuscript.
Correspondence to: Elchanan Nussinson, MD, Gastroenterology Institute, Emek Medical Center, Duchifat 7 st’, Afula 18101, Israel.
Telephone: +972-5-44943922 Fax: +972-4-6495314
Received: July 8, 2013
Revised: September 2, 2013
Accepted: September 16, 2013
Published online: January 21, 2014

A 77-year-old man with inflammatory bowel disease (IBD) and who was treated with anti-tumor necrosis factor (TNF), 6-mercaptopurine and corticosteroids, presented with primary effusion lymphoma-like lymphoma (PEL-like lymphoma) with massive ascites. The patient’s clinical course was complicated by acute renal insufficiency and hypotension, which led to death within 2 wk. In general, patients with IBD may have an increased risk for development of lymphoma, which is frequently associated with immunosuppressive and/or anti-TNF antibody therapies. PEL is a rare subset of lymphoma localized to serous body cavities, lacks tumor mass or nodal involvement, and is associated with infection by human herpes virus 8 (HHV-8). Primary neoplastic effusion may also be present in patients with large B-cell lymphoma without evidence of human immunodeficiency virus or HHV-8 infections. This type of lymphoma is classified as PEL-like lymphoma. Both PEL and PEL-like lymphoma types have been reported in patients undergoing immunosuppressive therapy, but to the best of our knowledge, the case described herein represents the first PEL-like lymphoma occurring in a patient with IBD.

Keywords: Inflammatory bowel disease, Lymphoma, Primary effusion lymphoma, Primary effusion lymphoma-like lymphoma, Immunosuppressive therapy

Core tip: We report a case of primary effusion lymphoma (PEL)-like lymphoma in an elderly, male inflammatory bowel disease (IBD) patient on 6-mercaptopurine (6-MP) treatment. This rare lymphoma subtype is localized to serous body cavities without tumor mass formation or nodal involvement, and has been previously reported in individuals with hepatitis C virus or Epstein-Barr virus infection, in patients who underwent organ transplantation, and elderly patients This novel case in an IBD patient illustrates the importance of considering PEL-like lymphoma in IBD patients treated with 6-MP and anti-tumor necrosis factor antibodies who subsequently develop serous body cavity effusion.