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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.
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.
How 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.
How 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.
What 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.
What 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.
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.
Averages across everyone diagnosed, often years ago. A median is the middle of a group: half the people counted lived longer than the figure shown, and some lived far longer. Your stage, subtype, age, fitness and the treatment you receive matter more than the average, and the numbers are improving quickly.
Leukaemias, myeloma and MDS live in the marrow and blood; lymphomas grow in lymph nodes and spleen. The node stations are the disease map, not a route of spread, and staging counts them.
In lymphoma the node stations are the disease itself; staging (Ann Arbor / Lugano) counts how many regions and sides of the diaphragm are involved.
Same organ: High-grade B-cell lymphoma with MYC and BCL2 rearrangements (double-hit lymphoma), Mediastinal grey zone lymphoma, Primary effusion lymphoma, Plasmablastic lymphoma, T-cell/histiocyte-rich large B-cell lymphoma, EBV-positive diffuse large B-cell lymphoma, Primary large B-cell lymphoma of the testis, Gastric MALT lymphoma, Ocular adnexal MALT lymphoma, Extranodal NK/T-cell lymphoma, Adult T-cell leukaemia/lymphoma, ALK-positive anaplastic large cell lymphoma, ALK-negative anaplastic large cell lymphoma, Breast implant-associated anaplastic large cell lymphoma, Primary cutaneous anaplastic large cell lymphoma, Lymphomatoid papulosis, Mycosis fungoides, Monomorphic epitheliotropic intestinal T-cell lymphoma, Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT lymphoma), Splenic marginal zone lymphoma, Nodal marginal zone lymphoma, Primary cutaneous marginal zone lymphoma, Primary cutaneous follicle centre lymphoma, Sezary syndrome, Nodal T-follicular helper cell lymphoma, angioimmunoblastic type (angioimmunoblastic T-cell lymphoma), Hepatosplenic T-cell lymphoma, Intravascular large B-cell lymphoma, Lymphomatoid granulomatosis, T-cell prolymphocytic leukaemia, Splenic B-cell lymphoma/leukaemia with prominent nucleoli (formerly B-cell prolymphocytic leukaemia and hairy cell leukaemia variant), T-cell large granular lymphocytic leukaemia, Mixed-phenotype acute leukaemia, Myeloid leukaemia of Down syndrome, Burkitt leukaemia, Marginal zone lymphoma, Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome), Primary mediastinal (thymic) large B-cell lymphoma, Leukaemia (all types), Acute myeloid leukaemia, Acute lymphoblastic leukaemia, Chronic lymphocytic leukaemia, Chronic myeloid leukaemia (CML), Diffuse large B-cell lymphoma, Follicular lymphoma, Hodgkin lymphoma, Mantle cell lymphoma, Multiple myeloma, Non-Hodgkin lymphoma (all types), Myelodysplastic syndromes / neoplasms (MDS), Myeloproliferative neoplasms (PV, ET, myelofibrosis), Polycythaemia vera (PV), Essential thrombocythaemia (ET), Waldenström macroglobulinaemia, Hairy cell leukaemia, Peripheral T-cell lymphomas (including cutaneous T-cell lymphoma), Blastic plasmacytoid dendritic cell neoplasm (BPDCN), Burkitt lymphoma, HIV-associated (AIDS-related) lymphomas, Chronic myelomonocytic leukaemia and MDS/MPN overlap neoplasms, Systemic mastocytosis, Erdheim-Chester disease, Rosai-Dorfman disease and other histiocytic neoplasms, Langerhans cell histiocytosis (LCH), Post-transplant lymphoproliferative disorder (PTLD), FLT3-mutated acute myeloid leukaemia, IDH1- and IDH2-mutated acute myeloid leukaemia, NPM1-mutated and KMT2A-rearranged acute myeloid leukaemia, Secondary and therapy-related acute myeloid leukaemia, Acute promyelocytic leukaemia, Acute myeloid leukaemia in older or unfit patients, Smouldering multiple myeloma, Newly diagnosed multiple myeloma, transplant-eligible, Newly diagnosed multiple myeloma, transplant-ineligible, Relapsed or refractory multiple myeloma, Plasma cell leukaemia, Lower-risk myelodysplastic syndromes, Higher-risk myelodysplastic syndromes, Chronic lymphocytic leukaemia, first treatment, Relapsed or refractory chronic lymphocytic leukaemia, Richter transformation of chronic lymphocytic leukaemia, Chronic myeloid leukaemia, chronic phase, Chronic myeloid leukaemia, accelerated and blast phase, Primary myelofibrosis, Standard-risk B-cell acute lymphoblastic leukaemia in children, High-risk acute lymphoblastic leukaemia in children (high-risk B-ALL and T-ALL), Philadelphia chromosome-positive acute lymphoblastic leukaemia in children (Ph-positive ALL), Philadelphia chromosome-like acute lymphoblastic leukaemia (Ph-like or BCR::ABL1-like ALL), Infant acute lymphoblastic leukaemia (KMT2A-rearranged, under one year), Relapsed and refractory acute lymphoblastic leukaemia in children, Acute myeloid leukaemia in children, Erdheim-Chester disease, Rosai-Dorfman-Destombes disease, Single-system Langerhans cell histiocytosis (bone, skin or one other organ), Multisystem Langerhans cell histiocytosis (with or without risk-organ involvement), Indolent and smouldering systemic mastocytosis, Advanced systemic mastocytosis (aggressive SM, SM with an associated haematological neoplasm, mast cell leukaemia), Early-stage classical Hodgkin lymphoma (stage I to II), Advanced-stage classical Hodgkin lymphoma (stage III to IV), Nodular lymphocyte-predominant Hodgkin lymphoma (nodular lymphocyte-predominant B-cell lymphoma), Relapsed and refractory classical Hodgkin lymphoma
No subtypes recorded beyond the ones named in the family strip above.
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.
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.
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.
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Query for this cancer: (TITLE:"Enteropathy-associated T-cell lymphoma" OR ABSTRACT:"Enteropathy-associated T-cell lymphoma" OR TITLE:"EATL" OR ABSTRACT:"EATL" OR TITLE:"Enteropathy-type T-cell lymphoma" OR ABSTRACT:"Enteropathy-type T-cell lymphoma" OR TITLE:"Enteropathy-associated and hepatosplenic T-cell lymphoma" OR ABSTRACT:"Enteropathy-associated and hepatosplenic T-cell lymphoma" OR TITLE:"Coeliac-associated T-cell lymphoma" OR ABSTRACT:"Coeliac-associated T-cell lymphoma" OR TITLE:"Type I EATL" OR ABSTRACT:"Type I EATL") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Enteropathy-associated T-cell lymphoma, not a curated reading list.
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.
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.
The International Consensus Classification lists type II refractory coeliac disease as a provisional entity; WHO-HAEM5 does not list it separately.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Reduce to 75% for CrCl 15-50.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
See all on the product pages:EtoposideIfosfamideMethotrexate·Printable cards in the navigator
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