{"entity":{"id":"enteropathy-associated-t-cell-lymphoma","kind":"cancer","name":"Enteropathy-associated T-cell lymphoma","aka":["EATL","Enteropathy-type T-cell lymphoma","Enteropathy-associated and hepatosplenic T-cell lymphoma","Coeliac-associated T-cell lymphoma","Type I EATL"],"tldr":"An aggressive T-cell lymphoma of the small bowel that arises out of coeliac disease, usually in somebody whose coeliac disease was diagnosed late or has not responded to a gluten-free diet. It often announces itself as a perforation or obstruction of the bowel in a person who is already underweight, which is why treatment has to deal with nutrition at the same time as the lymphoma.","summary":"What it is. A lymphoma of the T cells that live between the cells lining the small bowel. It arises in people with coeliac disease, the immune reaction to gluten, and particularly in those whose disease was diagnosed in adulthood or whose symptoms have not settled on a gluten-free diet. It is the reason coeliac disease that stops responding to the diet is investigated rather than managed.\n\nHow it presents, and why that shapes the treatment. WHO-HAEM5 tabulates the presentation: abdominal symptoms, with perforation or obstruction of the bowel common, deep involvement of the bowel wall, and a tumour made of pleomorphic large or medium cells against a prominent inflammatory background. A substantial proportion of patients come to attention as a surgical emergency. They are usually malnourished before the lymphoma starts, because the coeliac disease has been damaging the bowel, and chemotherapy in a malnourished person with a bowel at risk of perforating is a different proposition from chemotherapy in a well person. Nutritional support, often intravenous, and surgical involvement are part of the treatment and not an afterthought.\n\nHow it differs from the lymphoma it is most often confused with. Monomorphic epitheliotropic intestinal T-cell lymphoma arises in the same part of the gut and presents in the same way, and it is not associated with coeliac disease. Under the microscope it is monotonous where this one is pleomorphic, its cells are usually CD8-positive where these are usually negative for both CD4 and CD8, and it carries SETD2 mutations which this does not. It was called type II enteropathy-associated T-cell lymphoma until 2016 and has had its own name and its own page since.\n\nWhat treatment achieves. The Newcastle group reported both halves of the picture from one population. Treated with conventional anthracycline-based chemotherapy, with or without surgery, median progression-free survival was 3.4 months and median overall survival 7.1 months. From 1998 the same group gave patients fit enough for it a regimen of ifosfamide, etoposide and epirubicin alternating with methotrexate, followed by an autologous stem cell transplant; in 26 patients treated that way, five-year progression-free survival was 52 per cent and overall survival 60 per cent, significantly better than the historical comparison. That is a before-and-after comparison within one region rather than a randomised trial, and it is the best evidence this disease has.\n\nWhat should also happen. A strict gluten-free diet is continued, both for the bowel and because the rest of the family may need testing for coeliac disease. Refractory coeliac disease of the type with an abnormal clone of lymphocytes is the precursor state, and the International Consensus Classification lists it as a provisional entity of its own, which WHO-HAEM5 does not.","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":"Evaluation of enteropathy-associated T-cell lymphoma comparing standard therapies with a novel regimen including autologous stem cell transplantation (Sieniawski, Blood 2010)","url":"https://doi.org/10.1182/blood-2009-07-231324"},{"label":"Lymphoma incidence, survival and prevalence 2004 to 2014, subtype analyses from the UK Haematological Malignancy Research Network (Smith, Br J Cancer 2015)","url":"https://doi.org/10.1038/bjc.2015.94"},{"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":["monomorphic-epitheliotropic-intestinal-t-cell-lymphoma","peripheral-t-cell-lymphoma","hepatosplenic-t-cell-lymphoma","small-bowel","non-hodgkin-lymphoma"],"cancers":[],"sections":[],"technologies":["histopathology-ihc","fdg-pet","autologous-stem-cell-transplant"],"targets":[],"drugs":["ifosfamide","etoposide","methotrexate"],"companies":[],"institutions":[],"pathways":[],"terms":["lymphoma-nodal-versus-extranodal","lymphoma-classification-2022","lymphoma-pit-score","lymphoma-tx-transplant-role","lymphoma-lugano-gastrointestinal"],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"haematologic","burden":"Rare. In the population-based series from the Scotland and Newcastle Lymphoma Group, the overall incidence was 0.14 cases per 100,000 people a year, and 54 patients were identified over a five-year period. In the United Kingdom population series that reports lymphoma by subtype, 24 of 5,796 lymphomas were of the enteropathy type, a European age-standardised rate of 0.08 per 100,000 a year, a median age at diagnosis of 62.7 years and five-year relative survival of 28.0 per cent.","subtypes":[],"biomarkers":["Coeliac disease, established or newly discovered at the time of the lymphoma, which is part of the definition","A T-cell phenotype that is most often negative for both CD4 and CD8, with CD30 often positive","Pleomorphic large or medium-sized cells against a prominent inflammatory background, which separates it from the monomorphic intestinal lymphoma","Gains of 9q34 and loss of 16q12, with mutations of the JAK-STAT pathway, commonly JAK1 and STAT3","Absence of Epstein-Barr virus","Nutritional state, which determines what treatment is possible"],"standardOfCare":[{"setting":"Diagnosis, and the coeliac disease behind it","approach":"The diagnosis is often made on bowel resected as an emergency for perforation or obstruction. Where there is time, it is made at endoscopy with biopsies of the small bowel. Coeliac disease is confirmed or newly diagnosed at the same time, and the rest of the family is offered testing. Staging uses the gastrointestinal system that counts depth and node involvement, and imaging of the whole abdomen matters because disease is often multifocal.","refs":["endoscopy","histopathology-ihc","lymphoma-lugano-gastrointestinal","fdg-pet"],"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":"Nutrition and the surgical risk, which come first","approach":"Most patients are malnourished before the lymphoma starts, because the coeliac disease has been damaging the bowel, and the bowel is at risk of perforating during treatment. Nutritional assessment and support, often intravenous, and early involvement of a surgeon are part of the treatment rather than an afterthought, and they determine what chemotherapy is possible. A strict gluten-free diet is continued throughout.","refs":["lymphoma-tx-regimen-alphabet","peripheral-t-cell-lymphoma"],"guideline":{"version":"NCI PDQ: adult non-Hodgkin lymphoma treatment","url":"https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq"}},{"setting":"Systemic treatment","approach":"In the population-based series from northern England and Scotland, conventional anthracycline-based chemotherapy with or without surgery gave a median progression-free survival of 3.4 months and overall survival of 7.1 months in 54 patients. From 1998 the same group gave patients fit enough for it ifosfamide, etoposide and epirubicin alternating with methotrexate, followed by an autologous stem cell transplant; in 26 patients treated that way, five-year progression-free survival was 52 per cent and overall survival 60 per cent. That is a comparison against a historical group from the same region rather than a randomised trial, and it is the best evidence this disease has. A clinical trial is a reasonable first choice.","refs":["ifosfamide","etoposide","methotrexate","autologous-stem-cell-transplant","lymphoma-tx-transplant-role","lymphoma-tx-regimen-alphabet"],"guideline":{"version":"Scotland and Newcastle Lymphoma Group, Blood 2010; NCCN T-Cell Lymphomas; NCI PDQ","url":"https://doi.org/10.1182/blood-2009-07-231324"}}],"stateOfArt":[],"history":[{"year":2010,"title":"A population picture and a regimen that improved it","note":"Among 54 patients identified in northern England and Scotland, incidence was 0.14 per 100,000 a year and conventional chemotherapy gave a median progression-free survival of 3.4 months and overall survival of 7.1 months; 26 patients given ifosfamide, etoposide and epirubicin with methotrexate followed by autologous transplant had five-year progression-free survival of 52 per cent and overall survival of 60 per cent.","refs":["autologous-stem-cell-transplant","ifosfamide","etoposide","methotrexate"]},{"year":2016,"title":"Split in two","note":"The revised fourth edition of the WHO classification separated the type that is not associated with coeliac disease and gave it its own name, monomorphic epitheliotropic intestinal T-cell lymphoma.","refs":[]},{"year":2022,"title":"The precursor state recognised by one classification","note":"The International Consensus Classification lists type II refractory coeliac disease as a provisional entity; WHO-HAEM5 does not list it separately.","refs":["lymphoma-classification-2022"]}],"pipeline":[],"openProblems":["No randomised trial has been run in this disease. The regimen with the best results was compared against a historical group from the same region.","Many patients present as a surgical emergency in a malnourished state, so a substantial proportion are never well enough to receive the treatment that works best.","Whether earlier diagnosis of coeliac disease and better adherence to a gluten-free diet prevent this lymphoma has not been shown, although it is the usual assumption."],"parent":"peripheral-t-cell-lymphoma"},"route":"/cancers/enteropathy-associated-t-cell-lymphoma/","neighbours":{"cancer":[{"id":"hepatosplenic-t-cell-lymphoma","kind":"cancer","name":"Hepatosplenic T-cell lymphoma","route":"/cancers/hepatosplenic-t-cell-lymphoma/"},{"id":"monomorphic-epitheliotropic-intestinal-t-cell-lymphoma","kind":"cancer","name":"Monomorphic epitheliotropic intestinal T-cell lymphoma","route":"/cancers/monomorphic-epitheliotropic-intestinal-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":"autologous-stem-cell-transplant","kind":"technology","name":"Autologous stem cell transplant (high-dose therapy)","route":"/technologies/autologous-stem-cell-transplant/"},{"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":"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/"}],"term":[{"id":"endoscopy","kind":"term","name":"Endoscopy (EGD, EUS, ERCP)","route":"/terms/endoscopy/"},{"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-transplant-role","kind":"term","name":"Stem cell transplant in lymphoma: what it is still for","route":"/terms/lymphoma-tx-transplant-role/"},{"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/"}]}}