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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.
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.
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 corpus does not quote a geographic distribution for it, because it could not verify one.
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, Enteropathy-associated 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.
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.
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.
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Query for this cancer: (TITLE:"Monomorphic epitheliotropic intestinal T-cell lymphoma" OR ABSTRACT:"Monomorphic epitheliotropic intestinal T-cell lymphoma" OR TITLE:"MEITL" OR ABSTRACT:"MEITL" OR TITLE:"Type II enteropathy-associated T-cell lymphoma" OR ABSTRACT:"Type II enteropathy-associated T-cell lymphoma" OR TITLE:"Type II EATL" OR ABSTRACT:"Type II EATL" OR TITLE:"Monomorphic CD56-positive intestinal T-cell lymphoma" OR ABSTRACT:"Monomorphic CD56-positive intestinal T-cell lymphoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Monomorphic epitheliotropic intestinal T-cell lymphoma, not a curated reading list.
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.
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.
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.
The targets of this cancer's medicines and the ones linked to it directly.
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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.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Fatal if given intrathecally: label all syringes.
Reduce to 75% for CrCl 15-50.
High-dose methotrexate requires normal renal function, hydration, urine alkalinisation and leucovorin rescue with level monitoring.
Reduce by 50% for bilirubin 20-50 µmol/L and 75% for 50-85 µmol/L.
See all on the product pages:CyclophosphamideDoxorubicinEtoposideIfosfamideMethotrexateVincristine·Printable cards in the navigator
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