Hepatosplenic T-Cell Lymphoma: A Rare Complication of Monotherapy with Thiopurines in Crohn’s Disease

• Published in: GE Portuguese journal of gastroenterology Vol. 26; no. 4; pp. 279 - 284
• Main Authors: Carvão, Joana, Magno Pereira, Vítor, Jacinto, Fernando, Sousa Andrade, Carla, Jasmins, Luís
• Format: Journal Article
• Language: English
• Published: Basel, Switzerland S. Karger AG 01-07-2019; Karger Publishers
• Subjects: Abdomen; Anemia; Biopsy; Blood; Bone marrow; Case reports; Chemotherapy; Clinical Case Study; Crohn’s disease; Fever; Gastroenterology; Hepatosplenic T-cell lymphoma; Inflammatory bowel disease; Literature reviews; Lymphoma; Patients; Sepsis; Stains & staining; Staphylococcus infections; Thiopurines; Tomography; Tumor necrosis factor-TNF; Hepatosplenic T-cell lymphoma; Thiopurines; Crohn’s disease
• ISSN: 2341-4545; 2387-1954
• DOI: 10.1159/000493350

Hepatosplenic T-cell lymphoma (HSTCL) is an extremely rare and aggressive form of non-Hodgkin lymphoma associated with poor response to treatment and high mortality. There is an increased incidence among patients with inflammatory bowel disease, especially young male patients under 35 years old and on combination therapy (thiopurine and anti-TNF-α). We describe a case of HSTCL in a young male patient with stenosing ileal Crohn’s disease on azathioprine monotherapy for 4.8 years admitted to our hospital with intra- abdominal sepsis. Despite chemotherapy, the patient eventually died 1 month after the diagnosis. Through a literature review, we identified 18 additional cases of HSTCL in Crohn’s disease patients that had only been treated with thiopurine monotherapy. The authors intend to highlight the rarity of this diagnosis especially with azathioprine monotherapy and the diagnostic challenge in a case that presented with intra-abdominal sepsis.