Hodgkin lymphoma of the gastrointestinal tract in patients with inflammatory bowel disease: Portrait of a rare clinical entity. (August 2018)

• Record Type: Journal Article
• Authors: Barzilai, Merav; Polliack, Aaron; Avivi, Irit; Herishanu, Yair; Ram, Ron; Tang, Catherine; Perry, Chava; Sarid, Nadav
• Is Part Of: Leukemia research. Volume 71(2018)
• Page Start: 1
• Page End: 5
• Publication Date: 2018-08
• Subjects: Inflammatory bowel disease -- Hodgkin lymphoma -- Gastrointestinal; Leukemia -- Periodicals; Leukemia -- Periodicals; Leucémie -- Périodiques; Leukemia; Periodicals; Electronic journals; Electronic journals; 616.9941905
• DOI: 10.1016/j.leukres.2018.06.008
• Languages: English
• ISSNs: 0145-2126

Abstract

Highlights: Patients with IBD who develop gastrointestinal HL have distinct characteristics. A striking male preponderance (male to female ratio of 4:1). Crohn's disease being predominant with preference to develop in inflamed sites. EBV positivity and mixed cellularity histology. A unique pattern of spread to the liver. Abstract: Patients with inflammatory bowel disease (IBD) on immunosuppression are at risk of developing lymphoma, particularly primary gastrointestinal (GI) tract non-Hodgkin lymphoma. Primary GI Hodgkin lymphoma (HL) in this setting, however, is rare and poorly defined. Here we review the available literature and also report a patient with Crohn's disease (CD) who developed GI HL. Our search yielded 12 single case studies and 7 case series involving 22 patients published between 1978–2016. Twenty-one (91%) patients had CD, and 2 had ulcerative colitis. The median age at lymphoma diagnosis was 39 years, and 18 (78%) patients were males. HL was diagnosed at a median of 8 years after IBD detection and 2 years after commencing immunosuppression. HL had a predilection (80%) to involve the inflamed GI site and the histological subtype was mixed cellularity in 65% of cases. In-situ hybridization for Epstein-Barr virus (EBV)-encoded RNA was positive in all documented cases. HL was diagnosed in stages I, II, IV in 35%, 20% and 45% of the patients, respectively. Notably, 66% of patients with advanced disease had liver involvement. Immunosuppression was stopped in most (69%) patients at HL diagnosis. Treatment used was either chemotherapy only, surgery followed by chemotherapy, or surgery alone in 50%, 33% and 16% of cases, respectively. Four patients had an IBD flare during HL remission. Patients with IBD who develop GI HL have distinct characteristics; male sex, predominance of CD, preference to develop in inflamed sites, mixed cellularity histology, EBV positivity, and a unique spread to the liver pattern.