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Small intestinal enteropathy-associated T-cell lymphoma

MedGen UID:
275445
•Concept ID:
C1519371
•
Neoplastic Process
Synonyms: enteropathy-associated T-cell lymphoma of small intestine; Small Intestinal EATL; small intestinal EATL; Small Intestinal Enteropathy-Associated T-Cell Lymphoma; small intestinal enteropathy-associated T-cell lymphoma; Small Intestinal Enteropathy-Type T-Cell Lymphoma; small intestinal enteropathy-type T-cell lymphoma; small intestine enteropathy-associated T-cell lymphoma
 
Monarch Initiative: MONDO:0006418

Definition

An enteropathy-associated T-cell lymphoma arising from the small intestine, most commonly the jejunum or ileum. Patients usually present with abdominal pain, often associated with intestinal perforation. It is associated with celiac disease. The lymphoma cells are usually medium-sized to large and form an ulcerating mucosal lesion with invasion of the small intestinal wall. Villous atrophy is present in the adjacent small intestinal mucosa. In a minority of cases the lymphoma cells are medium-sized and form a monomorphic infiltrate. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSmall intestinal enteropathy-associated T-cell lymphoma

Professional guidelines

PubMed

AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review.
Green PHR, Paski S, Ko CW, Rubio-Tapia A
Gastroenterology 2022 Nov;163(5):1461-1469. Epub 2022 Sep 19 doi: 10.1053/j.gastro.2022.07.086. PMID: 36137844
Mechanisms and management of refractory coeliac disease.
van Gils T, Nijeboer P, van Wanrooij RL, Bouma G, Mulder CJ
Nat Rev Gastroenterol Hepatol 2015 Oct;12(10):572-9. Epub 2015 Sep 8 doi: 10.1038/nrgastro.2015.155. PMID: 26347156
Celiac disease: diagnosis and treatment.
Hvas CL, Jensen MD, Reimer MC, Riis LB, Rumessen JJ, Skovbjerg H, Teisner A, Wildt S
Dan Med J 2015 Apr;62(4):C5051. PMID: 25872537

Recent clinical studies

Etiology

Celiac Disease Is a Risk Factor for Mature T and NK Cell Lymphoma: A Mendelian Randomization Study.
Martín-Masot R, Herrador-López M, Navas-López VM, Carmona FD, Nestares T, Bossini-Castillo L
Int J Mol Sci 2023 Apr 13;24(8) doi: 10.3390/ijms24087216. PMID: 37108375Free PMC Article
New developments in non-Hodgkin lymphoid malignancies.
Ganapathi KA, Brown LE, Prakash S, Bhargava P
Pathology 2021 Apr;53(3):349-366. Epub 2021 Mar 5 doi: 10.1016/j.pathol.2021.01.002. PMID: 33685720
Refractory Celiac Disease.
Malamut G, Cellier C
Gastroenterol Clin North Am 2019 Mar;48(1):137-144. Epub 2018 Dec 13 doi: 10.1016/j.gtc.2018.09.010. PMID: 30711206
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Enteropathy associated T-cell lymphoma and its precursor lesions.
van de Water JM, Cillessen SA, Visser OJ, Verbeek WH, Meijer CJ, Mulder CJ
Best Pract Res Clin Gastroenterol 2010 Feb;24(1):43-56. doi: 10.1016/j.bpg.2009.11.002. PMID: 20206108

Diagnosis

AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review.
Green PHR, Paski S, Ko CW, Rubio-Tapia A
Gastroenterology 2022 Nov;163(5):1461-1469. Epub 2022 Sep 19 doi: 10.1053/j.gastro.2022.07.086. PMID: 36137844
Refractory Celiac Disease.
Malamut G, Cellier C
Gastroenterol Clin North Am 2019 Mar;48(1):137-144. Epub 2018 Dec 13 doi: 10.1016/j.gtc.2018.09.010. PMID: 30711206
Rare T-Cell Subtypes.
van der Weyden C, McCormack C, Lade S, Johnstone RW, Prince HM
Cancer Treat Res 2019;176:195-224. doi: 10.1007/978-3-319-99716-2_10. PMID: 30596220
Refractory celiac disease.
Rishi AR, Rubio-Tapia A, Murray JA
Expert Rev Gastroenterol Hepatol 2016;10(4):537-46. Epub 2015 Dec 16 doi: 10.1586/17474124.2016.1124759. PMID: 26603931
Enteropathy-associated T-cell lymphoma.
Ferreri AJ, Zinzani PL, Govi S, Pileri SA
Crit Rev Oncol Hematol 2011 Jul;79(1):84-90. Epub 2010 Jul 23 doi: 10.1016/j.critrevonc.2010.06.006. PMID: 20655757

Therapy

Celiac Disease Is a Risk Factor for Mature T and NK Cell Lymphoma: A Mendelian Randomization Study.
Martín-Masot R, Herrador-López M, Navas-López VM, Carmona FD, Nestares T, Bossini-Castillo L
Int J Mol Sci 2023 Apr 13;24(8) doi: 10.3390/ijms24087216. PMID: 37108375Free PMC Article
Refractory Celiac Disease.
Malamut G, Cellier C
Gastroenterol Clin North Am 2019 Mar;48(1):137-144. Epub 2018 Dec 13 doi: 10.1016/j.gtc.2018.09.010. PMID: 30711206
T-cell repertoires in refractory coeliac disease.
Ritter J, Zimmermann K, Jöhrens K, Mende S, Seegebarth A, Siegmund B, Hennig S, Todorova K, Rosenwald A, Daum S, Hummel M, Schumann M
Gut 2018 Apr;67(4):644-653. Epub 2017 Feb 10 doi: 10.1136/gutjnl-2016-311816. PMID: 28188172Free PMC Article
Refractory celiac disease.
Rishi AR, Rubio-Tapia A, Murray JA
Expert Rev Gastroenterol Hepatol 2016;10(4):537-46. Epub 2015 Dec 16 doi: 10.1586/17474124.2016.1124759. PMID: 26603931
Malignant complications of coeliac disease.
Brousse N, Meijer JW
Best Pract Res Clin Gastroenterol 2005 Jun;19(3):401-12. doi: 10.1016/j.bpg.2005.02.002. PMID: 15925845

Prognosis

AGA Clinical Practice Update on Management of Refractory Celiac Disease: Expert Review.
Green PHR, Paski S, Ko CW, Rubio-Tapia A
Gastroenterology 2022 Nov;163(5):1461-1469. Epub 2022 Sep 19 doi: 10.1053/j.gastro.2022.07.086. PMID: 36137844
Small bowel T-cell lymphoma: a MEITL-ing diagnosis.
Mago S, Mavilia M, Forouhar F, Vaziri H
Clin J Gastroenterol 2021 Aug;14(4):1071-1083. Epub 2021 Apr 21 doi: 10.1007/s12328-021-01417-3. PMID: 33881752
Refractory Celiac Disease.
Malamut G, Cellier C
Gastroenterol Clin North Am 2019 Mar;48(1):137-144. Epub 2018 Dec 13 doi: 10.1016/j.gtc.2018.09.010. PMID: 30711206
Complications of coeliac disease.
Malamut G, Cellier C
Best Pract Res Clin Gastroenterol 2015 Jun;29(3):451-8. Epub 2015 May 14 doi: 10.1016/j.bpg.2015.05.005. PMID: 26060109
Enteropathy-associated T-cell lymphoma.
Ferreri AJ, Zinzani PL, Govi S, Pileri SA
Crit Rev Oncol Hematol 2011 Jul;79(1):84-90. Epub 2010 Jul 23 doi: 10.1016/j.critrevonc.2010.06.006. PMID: 20655757

Clinical prediction guides

Celiac Disease Is a Risk Factor for Mature T and NK Cell Lymphoma: A Mendelian Randomization Study.
Martín-Masot R, Herrador-López M, Navas-López VM, Carmona FD, Nestares T, Bossini-Castillo L
Int J Mol Sci 2023 Apr 13;24(8) doi: 10.3390/ijms24087216. PMID: 37108375Free PMC Article
Single-Cell Analysis of Refractory Celiac Disease Demonstrates Inter- and Intra-Patient Aberrant Cell Heterogeneity.
Dieckman T, Schreurs M, Mahfouz A, Kooy-Winkelaar Y, Neefjes-Borst A, Bouma G, Koning F
Cell Mol Gastroenterol Hepatol 2022;14(1):173-192. Epub 2022 Mar 23 doi: 10.1016/j.jcmgh.2022.03.005. PMID: 35338007Free PMC Article
T-cell repertoires in refractory coeliac disease.
Ritter J, Zimmermann K, Jöhrens K, Mende S, Seegebarth A, Siegmund B, Hennig S, Todorova K, Rosenwald A, Daum S, Hummel M, Schumann M
Gut 2018 Apr;67(4):644-653. Epub 2017 Feb 10 doi: 10.1136/gutjnl-2016-311816. PMID: 28188172Free PMC Article
Refractory celiac disease.
Rishi AR, Rubio-Tapia A, Murray JA
Expert Rev Gastroenterol Hepatol 2016;10(4):537-46. Epub 2015 Dec 16 doi: 10.1586/17474124.2016.1124759. PMID: 26603931
Small bowel imaging in celiac disease.
Van Weyenberg SJB, Mulder CJJ, Van Waesberghe JHTM
Dig Dis 2015;33(2):252-259. Epub 2015 Apr 22 doi: 10.1159/000369516. PMID: 25925931

Recent systematic reviews

Pancreatic Cancer in Celiac Disease Patients-A Systematic Review and Meta-Analysis.
Gromny I, Neubauer K
Int J Environ Res Public Health 2023 Jan 14;20(2) doi: 10.3390/ijerph20021565. PMID: 36674320Free PMC Article

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