Hepatosplenic T-cell lymphoma in a 47-year-old Crohn’s disease patient on thiopurine monotherapy

Abstract

Hepatosplenic T-cell lymphoma (HSTCL) is a rare non-Hodgkin lymphoma with a high mortality rate. Higher incidence is reported in patients with inflammatory bowel disease, specifically in male patients that are younger than 35 years, and have been treated with thiopurine and tumor necrosis factor (TNF)-α inhibitor combination therapy for over 2 years. In this case report we describe a 47-year-old patient with Crohn’s disease (CD) who developed HSTCL after having been treated with thiopurine monotherapy for 14 years. To our best knowledge, only eleven cases exist of patients with CD who developed HSTCL while on thiopurine monotherapy. We report the first patient with CD, older than 35 years, who developed HSTCL while on thiopurine monotherapy. This emphasizes that HSTCL risk is not limited to young men receiving both thiopurines and TNF-α inhibitors.

Keywords: Hepatosplenic T-cell lymphoma; Thiopurine; Crohn’s disease; Immunosuppression

Core tip: In this manuscript we provide an overview of all known cases in literature with Crohn’s disease (CD) who developed hepatosplenic T-cell lymphoma (HSTCL) while on thiopurine monotherapy. In addition, we present our case of a patient with CD on thiopurine monotherapy who developed HSTCL at a relatively old age. This emphasizes that HSTCL may develop at all ages, even when the patient is solely on thiopurine monotherapy.