Hepatosplenic T-cell lymphoma associated to thiopurine in Ulcerative Colitis patient from Brazil: A case report

Abstract

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and usually fatal peripheral T-cell lymphoma that primarily affects men younger than 35 years old. Treatment with thiopurines of patients with inflammatory bowel disease (IBD) has been associated with HSTCL, especially when submitted to a combined therapy with antibodies to tumor necrosis factor (anti-TNFs). Herein, we describe the case of an 25-year-old man with diagnosis of Ulcerative Colitis disease since 2003. He was initially treated with sulfasalazine 6g/dia, interspersed with prednisone during episodes of exacerbation. In July 2007 he started treatment with 50mg of azathioprine after a disease exacerbation. The dose was adjusted by the weight through 150mg/day, maintaining remission. Within 3 years and 3 months under azathioprine therapy he started to show changes in blood count and leukopenia. After an hematology evaluation, there was a diagnose of HSTCL. He died 8 months after diagnosis from chemotherapy-refractory lymphoma. Through a literature review, we identified 16 cases of HSTCL in patients with Inflammatory Bowel Disease treated with azathioprine or 6-mercaptopurine alone. Only 06 of these patients had the diagnosis of Ulcerative Colitis. The median age at diagnosis of HSTCL was 24 years (range, 15-35 years). The median duration of thiopurine exposure was 06 years (range, 3-17 years). 11 of these patients died, with only 04 patients achieving remission. Despite the known benefits in the treatment of IBD, additional research is needed to better understand the role of thiopurines and TNF-alpha inhibitors in promoting HSTCL. The absolute risk and the frequency of HSTCL is very low. With respect to safety, however, special caution in prescribing this therapy to male 35 years-old and younger IBD patients should be recommended, mainly during a period of 24 months or more.