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6 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.
An aggressive T-cell lymphoma of the small bowel that looks and presents much like the lymphoma that complicates coeliac disease but has no connection with coeliac disease at all. It was separated out of that diagnosis in 2016, it is made of monotonous small to medium cells, and it often presents with perforation or obstruction of the bowel.
What it is. A lymphoma of the T cells between the cells lining the small bowel, like enteropathy-associated T-cell lymphoma, and otherwise a different disease. The name describes it: monomorphic, because the cells are monotonous small to medium-sized cells rather than the varied large ones of its neighbour; epitheliotropic, because the cells invade the lining itself; intestinal, because that is where it lives.
How it differs from the lymphoma it was carved out of. WHO-HAEM5 sets the differences out side by side. There is no association with coeliac disease. The cells are usually CD8-positive rather than negative for both CD4 and CD8. Necrosis is usually absent where it may be present in the other. The mutations differ: both carry gains of 9q34 and loss of 16q12, but this one carries mutations of SETD2 and of JAK3 and STAT5B, while the other carries JAK1 and STAT3. Neither carries Epstein-Barr virus, which separates both from extranodal NK/T-cell lymphoma when that disease involves the gut.
| Setting | Approach | Guideline |
|---|---|---|
| Diagnosis, and telling it from its neighbour | Often made on small bowel resected as an emergency for perforation or obstruction. What separates it from enteropathy-associated T-cell lymphoma is the absence of coeliac disease, the monotonous small to medium cells rather than varied large ones, a CD8-positive and CD56-positive phenotype, and SETD2 mutation with JAK3 and STAT5B rather than JAK1 and STAT3. Neither carries Epstein-Barr virus, which separates both from NK/T-cell lymphoma of the gut. | not mapped |
| Systemic treatment, and how thin the evidence is | There is no randomised evidence of any kind. The largest real-world comparison took 50 patients who received systemic chemotherapy and compared a modified version of the Newcastle regimen developed for enteropathy-associated T-cell lymphoma, giving cyclophosphamide, doxorubicin, vincristine and prednisone alternating with ifosfamide, etoposide and epirubicin but leaving out the methotrexate, against ordinary chemotherapy of the same backbone: median progression-free survival 14.4 against 6.6 months and overall survival 28.7 against 11.7 months, with the regimen remaining an independent predictor of better progression-free survival after adjustment. The two-year cumulative incidence of relapse in the central nervous system was 12.1 per cent, which is what the omitted methotrexate had been intended to prevent. Entry into a trial is a reasonable first choice rather than a last resort. | not mapped |