# Monomorphic epitheliotropic intestinal T-cell lymphoma

Source: https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/  
OnCo record `monomorphic-epitheliotropic-intestinal-t-cell-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

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.

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.

How 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.

What 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.

What 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.

## Fields

- Kind: Cancer
- Last checked: 2026-09-29
- Also known as: MEITL; Type II enteropathy-associated T-cell lymphoma; Type II EATL; Monomorphic CD56-positive intestinal T-cell lymphoma
- Tags: heme; lymphoma; subtype-page
- 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.
- 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

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#overview
- Types and stages (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#what-it-is
- Symptoms and diagnosis (on the hub): How this cancer shows itself, how the diagnosis is confirmed, and the biomarkers clinicians test for. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#finding-it [6 biomarkers]
- Treatment (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#treating-it [2 settings, 1 decision with options]
- Trials and papers (on the hub): Trials recruiting now, the landmark trials, the trials held by this cancer's subtypes, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#evidence [3 milestones]
- Biology and targets (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#science [1 target]
- Countries and centres (own page): Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/where-you-are/
- Decisions and support (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/#living-with-it [10 questions, 6 red cards]
- Pipeline and open problems (own page): Everything in development, the medicines held by this cancer's subtypes, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/coming/ [7 medicines, 3 open problems]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma/data/ [25 connected records]

## Standard of care

- 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. ([Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/), [Enteropathy-associated T-cell lymphoma](https://onco.cc/cancers/enteropathy-associated-t-cell-lymphoma/), [Epstein-Barr virus (EBV) in cancer](https://onco.cc/terms/ebv-term/), [Staging a lymphoma of the stomach or bowel](https://onco.cc/terms/lymphoma-lugano-gastrointestinal/))
- 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. ([Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Vincristine](https://onco.cc/drugs/vincristine/), [Prednisone](https://onco.cc/drugs/prednisone/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Etoposide](https://onco.cc/drugs/etoposide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [CNS prophylaxis in aggressive B-cell lymphoma, and the evidence against it](https://onco.cc/terms/lymphoma-tx-cns-prophylaxis/), [The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest](https://onco.cc/terms/lymphoma-tx-regimen-alphabet/))

## Open problems

- 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.

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Enteropathy-associated_T-cell_lymphoma
- WHO Classification of Haematolymphoid Tumours, 5th edition: lymphoid neoplasms (Alaggio, Leukemia 2022): https://doi.org/10.1038/s41375-022-01620-2
- International Consensus Classification of Mature Lymphoid Neoplasms (Campo, Blood 2022): https://doi.org/10.1182/blood.2022015851
- Modified Newcastle regimen in monomorphic epitheliotropic intestinal T-cell lymphoma, a real-world cohort of 56 patients (EJHaem 2026): https://doi.org/10.1002/jha2.70400
- NCI PDQ: adult non-Hodgkin lymphoma treatment (health professional version): https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq

## Connected records

- cancers: [Enteropathy-associated T-cell lymphoma](https://onco.cc/cancers/enteropathy-associated-t-cell-lymphoma/), [Extranodal NK/T-cell lymphoma](https://onco.cc/cancers/extranodal-nk-t-cell-lymphoma/), [Non-Hodgkin lymphoma (all types)](https://onco.cc/cancers/non-hodgkin-lymphoma/), [Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma)](https://onco.cc/cancers/peripheral-t-cell-lymphoma/), [Small intestine cancer (small bowel adenocarcinoma)](https://onco.cc/cancers/small-bowel/)
- technologies: [FDG PET](https://onco.cc/technologies/fdg-pet/), [Histopathology & immunohistochemistry](https://onco.cc/technologies/histopathology-ihc/)
- drugs: [Cyclophosphamide](https://onco.cc/drugs/cyclophosphamide/), [Doxorubicin](https://onco.cc/drugs/doxorubicin/), [Etoposide](https://onco.cc/drugs/etoposide/), [Ifosfamide](https://onco.cc/drugs/ifosfamide/), [Methotrexate](https://onco.cc/drugs/methotrexate/), [Prednisone](https://onco.cc/drugs/prednisone/), [Vincristine](https://onco.cc/drugs/vincristine/)
- terms: [CNS prophylaxis in aggressive B-cell lymphoma, and the evidence against it](https://onco.cc/terms/lymphoma-tx-cns-prophylaxis/), [Epstein-Barr virus (EBV) in cancer](https://onco.cc/terms/ebv-term/), [Nodal and extranodal lymphoma](https://onco.cc/terms/lymphoma-nodal-versus-extranodal/), [Prognostic Index for T-cell lymphoma (PIT)](https://onco.cc/terms/lymphoma-pit-score/), [Staging a lymphoma of the stomach or bowel](https://onco.cc/terms/lymphoma-lugano-gastrointestinal/), [The lymphoma regimen alphabet: R-CHOP, pola-R-CHP, DA-EPOCH-R, ABVD, BEACOPP and the rest](https://onco.cc/terms/lymphoma-tx-regimen-alphabet/), [The two lymphoma classifications of 2022 (WHO-HAEM5 and ICC)](https://onco.cc/terms/lymphoma-classification-2022/)

---
JSON: https://onco.cc/api/v1/entities/monomorphic-epitheliotropic-intestinal-t-cell-lymphoma.json