{"entity":{"id":"monomorphic-epitheliotropic-intestinal-t-cell-lymphoma","kind":"cancer","name":"Monomorphic epitheliotropic intestinal T-cell lymphoma","aka":["MEITL","Type II enteropathy-associated T-cell lymphoma","Type II EATL","Monomorphic CD56-positive intestinal T-cell lymphoma"],"tldr":"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.","summary":"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.\n\nHow 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.\n\nHow it presents. As abdominal pain, weight loss and often perforation or obstruction of the small bowel, deep in the bowel wall. Patients commonly come to attention through emergency surgery, and the diagnosis is made on the resected bowel.\n\nWhat is known about treating it. Less than for its neighbour. The largest real-world cohort 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 CHOP-based chemotherapy, in 50 patients who received systemic treatment. Median progression-free survival was 14.4 months against 6.6 and median overall survival 28.7 against 11.7 months, and the regimen remained an independent predictor of better progression-free survival after adjustment. The same study measured what the omitted methotrexate was meant to prevent: the two-year cumulative incidence of relapse in the central nervous system was 12.1 per cent. It is a retrospective comparison, not a trial.\n\nWhat a reader should take from this. This is a disease that was given its own name nine years ago, has no randomised evidence at all, and whose best available treatment comparison is a retrospective cohort of 50 treated patients. Entry into a trial is a reasonable first choice rather than a last resort, and the pages in this family say so where it is true.","asOf":"2026-09-29","wikipedia":"https://en.wikipedia.org/wiki/Enteropathy-associated_T-cell_lymphoma","links":[{"label":"WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022)","url":"https://doi.org/10.1038/s41375-022-01620-2"},{"label":"International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022)","url":"https://doi.org/10.1182/blood.2022015851"},{"label":"Modified Newcastle regimen in monomorphic epitheliotropic intestinal T-cell lymphoma, a real-world cohort of 56 patients (EJHaem 2026)","url":"https://doi.org/10.1002/jha2.70400"},{"label":"NCI PDQ: adult non-Hodgkin lymphoma treatment (health professional version)","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}],"tags":["heme","lymphoma","subtype-page"],"related":["enteropathy-associated-t-cell-lymphoma","peripheral-t-cell-lymphoma","extranodal-nk-t-cell-lymphoma","small-bowel","non-hodgkin-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","fdg-pet"],"targets":[],"drugs":["ifosfamide","etoposide","cyclophosphamide"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-nodal-versus-extranodal","lymphoma-classification-2022","lymphoma-pit-score","lymphoma-tx-cns-prophylaxis","lymphoma-lugano-gastrointestinal"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"Rare, and not reported separately in the United Kingdom population series, which predates the 2016 separation and counts it with enteropathy-associated T-cell lymphoma under the heading enteropathy type. The largest published real-world cohort assembled 56 patients diagnosed between 2002 and 2025 across several centres, and reports the median overall survival described in the literature as 7 to 15 months. The corpus does not quote a geographic distribution for it, because it could not verify one.","subtypes":[],"biomarkers":["Monotonous small to medium-sized cells invading the lining of the bowel, which is the name and the diagnosis","A CD8-positive, CD56-positive phenotype in most cases","SETD2 mutation, and mutations of JAK3 and STAT5B, which separate it from enteropathy-associated T-cell lymphoma","Gains of 9q34 and loss of 16q12, shared with enteropathy-associated T-cell lymphoma","Absence of coeliac disease, which is the main clinical difference","Absence of Epstein-Barr virus, which separates it from NK/T-cell lymphoma of the gut"],"standardOfCare":[{"setting":"Diagnosis, and telling it from its neighbour","approach":"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.","refs":["histopathology-ihc","enteropathy-associated-t-cell-lymphoma","ebv-term","lymphoma-lugano-gastrointestinal"],"guideline":{"version":"WHO Classification of Haematolymphoid Tumours, 5th edition (2022), with the International Consensus Classification (2022) where they differ","url":"https://doi.org/10.1038/s41375-022-01620-2"}},{"setting":"Systemic treatment, and how thin the evidence is","approach":"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.","refs":["cyclophosphamide","doxorubicin","vincristine","prednisone","ifosfamide","etoposide","methotrexate","lymphoma-tx-cns-prophylaxis","lymphoma-tx-regimen-alphabet"],"guideline":{"version":"Real-world cohort of 56 patients (EJHaem 2026); NCCN T-Cell Lymphomas; NCI PDQ","url":"https://doi.org/10.1002/jha2.70400"}}],"stateOfArt":[],"history":[{"year":2016,"title":"Given its own name","note":"The revised fourth edition of the WHO classification separated what had been called type II enteropathy-associated T-cell lymphoma and named it monomorphic epitheliotropic intestinal T-cell lymphoma, because it has no association with coeliac disease and differs in appearance and genetics.","refs":[]},{"year":2022,"title":"Kept as an entity, with its differences tabulated","note":"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.","refs":["lymphoma-classification-2022"]},{"year":2026,"title":"A real-world comparison of two regimens","note":"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.","refs":["ifosfamide","etoposide","cyclophosphamide"]}],"pipeline":[],"openProblems":["There is no randomised evidence of any kind in this disease, and the best comparison available is a retrospective cohort of 50 treated patients.","Whether methotrexate should be included to prevent relapse in the central nervous system is unresolved: the regimen that performed better left it out, and the two-year risk of relapse in the brain or spinal cord was still 12.1 per cent.","Because it is diagnosed on resected bowel after emergency surgery in many cases, the population that reaches systemic treatment is selected, and the published survival figures reflect that."],"parent":"peripheral-t-cell-lymphoma"},"route":"/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/","neighbours":{"cancer":[{"id":"enteropathy-associated-t-cell-lymphoma","kind":"cancer","name":"Enteropathy-associated T-cell lymphoma","route":"/cancers/enteropathy-associated-t-cell-lymphoma/"},{"id":"extranodal-nk-t-cell-lymphoma","kind":"cancer","name":"Extranodal NK/T-cell lymphoma","route":"/cancers/extranodal-nk-t-cell-lymphoma/"},{"id":"non-hodgkin-lymphoma","kind":"cancer","name":"Non-Hodgkin lymphoma (all types)","route":"/cancers/non-hodgkin-lymphoma/"},{"id":"peripheral-t-cell-lymphoma","kind":"cancer","name":"Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)","route":"/cancers/peripheral-t-cell-lymphoma/"},{"id":"small-bowel","kind":"cancer","name":"Small intestine cancer (small bowel adenocarcinoma)","route":"/cancers/small-bowel/"}],"technology":[{"id":"fdg-pet","kind":"technology","name":"FDG PET","route":"/technologies/fdg-pet/"},{"id":"histopathology-ihc","kind":"technology","name":"Histopathology & immunohistochemistry","route":"/technologies/histopathology-ihc/"}],"drug":[{"id":"cyclophosphamide","kind":"drug","name":"Cyclophosphamide","route":"/drugs/cyclophosphamide/"},{"id":"doxorubicin","kind":"drug","name":"Doxorubicin","route":"/drugs/doxorubicin/"},{"id":"etoposide","kind":"drug","name":"Etoposide","route":"/drugs/etoposide/"},{"id":"ifosfamide","kind":"drug","name":"Ifosfamide","route":"/drugs/ifosfamide/"},{"id":"methotrexate","kind":"drug","name":"Methotrexate","route":"/drugs/methotrexate/"},{"id":"prednisone","kind":"drug","name":"Prednisone","route":"/drugs/prednisone/"},{"id":"vincristine","kind":"drug","name":"Vincristine","route":"/drugs/vincristine/"}],"term":[{"id":"lymphoma-tx-cns-prophylaxis","kind":"term","name":"CNS prophylaxis in aggressive B-cell lymphoma, and the evidence against it","route":"/terms/lymphoma-tx-cns-prophylaxis/"},{"id":"ebv-term","kind":"term","name":"Epstein-Barr virus (EBV) in cancer","route":"/terms/ebv-term/"},{"id":"lymphoma-nodal-versus-extranodal","kind":"term","name":"Nodal and extranodal lymphoma","route":"/terms/lymphoma-nodal-versus-extranodal/"},{"id":"lymphoma-pit-score","kind":"term","name":"Prognostic Index for T-cell lymphoma (PIT)","route":"/terms/lymphoma-pit-score/"},{"id":"lymphoma-lugano-gastrointestinal","kind":"term","name":"Staging a lymphoma of the stomach or bowel","route":"/terms/lymphoma-lugano-gastrointestinal/"},{"id":"lymphoma-tx-regimen-alphabet","kind":"term","name":"The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest","route":"/terms/lymphoma-tx-regimen-alphabet/"},{"id":"lymphoma-classification-2022","kind":"term","name":"The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)","route":"/terms/lymphoma-classification-2022/"}]}}