Primary Gastrointestinal EBV-Associated Classical Hodgkin Lymphoma in Crohn Disease on Anti-TNF-α Therapy: A Rare Association

Background

Lymphomas present a significant challenge in the field of gastrointestinal diseases, often being mistaken for other gastrointestinal tumors or inflammatory bowel disease conditions, causing clinical confusion. Early diagnosis plays a pivotal role in effective treatment. This case highlights the importance of recognizing lymphoproliferative disorders as a rare association of anti-tumor necrosis factor-α (TNF-α) therapy.

Case Presentation

A 41-year-old man with a 15-year history of Crohn disease on long-term therapy with adalimumab underwent a right hemicolectomy due to a semi-circumferential lesion at the ileocecal valve causing near complete obstruction and severe anemia (Hgb 6.4 g/dL). Previous biopsies of the mass showed an Epstein Barr Virus-positive (EBV+) classic Hodgkin lymphoma (CHL) in Crohn disease. At resection, the lymphoma showed transmural involvement of the ileum and regional lymph nodes.

Conclusion

Primary intestinal CHL comprises less than 5% of gastrointestinal lymphomas; CHL arising in the context of Crohn disease is even more rare. Most lymphomas associated with inflammatory bowel disease and/or immunosuppression are non-Hodgkin type. In this case, the long-term treatment with anti-TNF-α and EBV positivity suggested an iatrogenic immunodeficiency-associated lymphoma, an emerging group of lymphoproliferative disorders associated with the increased use of immunosuppressants.