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Every dated change on the records linked to Monomorphic epitheliotropic intestinal T-cell lymphoma, newest first: approvals and regulatory steps on its medicines, trials that reported, guideline versions, milestones, and when this page itself was checked. Dates come from the records; none is inferred. Orientation, not medical advice.
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.
In 50 patients receiving systemic chemotherapy, a modified Newcastle regimen without methotrexate gave median progression-free survival of 14.4 months against 6.6 and overall survival of 28.7 against 11.7 months compared with CHOP-based chemotherapy; the two-year cumulative incidence of relapse in the central nervous system was 12.1 per cent.
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.
WHO-HAEM5 retained the entity and set out the features that separate the five T-cell and NK-cell conditions of the gut from each other, including the mutations that differ between this and enteropathy-associated T-cell lymphoma.