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4 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Hepatosplenic T-cell lymphoma is a rare, very aggressive lymphoma of young men in which gamma-delta T cells fill the liver, spleen and bone marrow without forming lumps in the nodes. It is linked to long-term immune suppression, above all thiopurines with or without anti-TNF drugs for inflammatory bowel disease, and is treated with intensive chemotherapy then a stem cell transplant where possible.
WHO-HAEM5 keeps hepatosplenic T-cell lymphoma as an entity of cytotoxic, usually gamma-delta T cells with sinusoidal infiltration of spleen, liver and marrow, isochromosome 7q and an aggressive course (Alaggio 2022). Whole-exome sequencing of 68 cases defined its drivers: chromatin-modifying genes (SETD2, INO80, ARID1B) mutated in 62 percent, SETD2 the most frequently silenced and shown to act as a tumour suppressor, and STAT5B (31 percent), STAT3 (9 percent) and PIK3CD (9 percent) mutations that activate targetable signalling (McKinney 2017). Of 36 patients with the lymphoma arising during treatment of inflammatory bowel disease, 20 had received infliximab with a thiopurine and 16 a thiopurine alone; 27 of 30 with known age were under 35 and only 2 of 31 were women (Clin Gastroenterol Hepatol 2011).
How it differs from its parent: no lymphadenopathy, a leukaemic and hepatosplenic pattern, a young male population, an iatrogenic immunosuppression association, and an outcome worse than most peripheral T-cell lymphomas, with median survival under two years in the older literature and CHOP alone rarely producing durable remission.
| Setting | Approach | Guideline |
|---|---|---|
| All cases | Intensive platinum- and cytarabine-based induction rather than CHOP, then allogeneic or autologous transplant in first remission for fit patients. | not mapped |