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HandWiki. Enteropathy-associated T-cell Lymphoma. Encyclopedia. Available online: https://encyclopedia.pub/entry/35070 (accessed on 25 September 2026).
HandWiki. Enteropathy-associated T-cell Lymphoma. Encyclopedia. Available at: https://encyclopedia.pub/entry/35070. Accessed September 25, 2026.
HandWiki. "Enteropathy-associated T-cell Lymphoma" Encyclopedia, https://encyclopedia.pub/entry/35070 (accessed September 25, 2026).
HandWiki. (2022, November 17). Enteropathy-associated T-cell Lymphoma. In Encyclopedia. https://encyclopedia.pub/entry/35070
HandWiki. "Enteropathy-associated T-cell Lymphoma." Encyclopedia. Web. 17 November, 2022.
Enteropathy-associated T-cell Lymphoma
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The content is sourced from: https://handwiki.org/wiki/Medicine:Enteropathy-associated_T-cell_lymphoma

Enteropathy-associated T-cell lymphoma (EATL), previously termed enteropathy-associated T-cell lymphoma, type I and at one time termed enteropathy-type T-cell lymphoma (ETTL), is a complication of coeliac disease in which a malignant T-cell lymphoma develops in areas of the small intestine affected by the disease's intense inflammation. While a relatively rare disease, it is the most common type of primary gastrointestinal T-cell lymphoma. EATL had been defined as a single type of small intestine lymphoma, but in 2008, the World Health Organization (WHO) divided the disease into two subtypes: 1) EATL type I, which occurs in individuals with coeliac disease, a chronic immune disorder causing inflammatory responses to dietary gluten primarily in the upper reaches (i.e. jejunum and duodenum) of the small intestine; and 2) EATL type II, a disorder similar to EATL type I that occurs without coeliac disease. While type I and II EATL share many similar features, post-2008 studies found some significant differences between the two types. In 2016, the WHO redefined the two diseases as separate entities, keeping the term enteropathy-associated T-cell lymphoma for the coeliac disease-associated lymphoma and terming type 2 disease as monomorphic epitheliotropic intestinal T cell lymphoma (MEITL). EATL is five to ten times more common than is MEITL. The WHO also defined a third type of intestinal T-cell lymphoma that cannot not be classified as EATL or MEITL as peripheral T-cell lymphoma not otherwise specified (ITCL-NOS). EATL arises from the malignant transformation of small-intestinal intraepithelial lymphocytes (IEL). IEL are a heterogeneous group of principally T-cell lymphocytes residing in epithelial tissues that interface the environment, such as the mucosa of the bronchi, reproductive tract and gastrointestinal tract. At these sites, IEL are exposed and regulate immune responses to non-dietary and dietary antigens, pathogenic and non-pathogenic organisms and injured self tissues. Gastrointestinal tract IEL appear in the epithelium of the small intestine, colon, stomach and esophagus, residing between the epithelial cells that line these organs' lumens. These IEL often exhibit natural killer and cytotoxic T-cell cell activation markers, contain various toxic agents (e.g. perforin, granzyme) and therefore are capable, if activated, of causing severe tissue injuries. With coeliac disease, the IEL react to the glutelins in dietary gluten by increasing their numbers, becoming pathologically active, producing chronic inflammation that injures intestinal cells, interfering with nutrient absorption and creating an environment conducive to their malignant transformation into EATL. Optimal treatment of EATL has used regimens consisting of intensive chemotherapy, hematopoietic stem cell transplantation and, in cases with bulky, obstructive and/or perforated bowel disease, surgical intervention. The disease has a five-year overall survival rate of only ~20%. However, recent studies focusing on the malignant IEL in EATL have increased understanding of the disease and suggested newer chemotherapy-based strategies and novel molecular targets that might be attacked therapeutically to improve the disease's prognosis.

glutelins dietary gluten coeliac disease

References

  1. "Complications of coeliac disease". Best Practice & Research. Clinical Gastroenterology 29 (3): 451–8. June 2015. doi:10.1016/j.bpg.2015.05.005. PMID 26060109.  https://dx.doi.org/10.1016%2Fj.bpg.2015.05.005
  2. "Celiac disease: a clinical review". Abdominal Radiology 42 (2): 351–360. February 2017. doi:10.1007/s00261-016-1034-y. PMID 28078381.  https://dx.doi.org/10.1007%2Fs00261-016-1034-y
  3. "Pathogenesis of Enteropathy-Associated T Cell Lymphoma". Current Hematologic Malignancy Reports 13 (4): 308–317. August 2018. doi:10.1007/s11899-018-0459-5. PMID 29943210.  https://dx.doi.org/10.1007%2Fs11899-018-0459-5
  4. "Enteropathy-Associated T-Cell Lymphoma". Current Hematologic Malignancy Reports 11 (6): 504–513. December 2016. doi:10.1007/s11899-016-0357-7. PMID 27900603.  https://dx.doi.org/10.1007%2Fs11899-016-0357-7
  5. "Human intraepithelial lymphocytes". Mucosal Immunology 11 (5): 1281–1289. September 2018. doi:10.1038/s41385-018-0016-5. PMID 29674648.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=6178824
  6. "Limited role of HLA DQ2/8 genotyping in diagnosing coeliac disease". Scottish Medical Journal 62 (1): 25–27. February 2017. doi:10.1177/0036933016689008. PMID 28084899.  https://dx.doi.org/10.1177%2F0036933016689008
  7. "Alleles Report < IMGT/HLA < IPD < EMBL-EBI". https://www.ebi.ac.uk/cgi-bin/ipd/imgt/hla/get_allele.cgi?DQB1*0501. 
  8. "Alleles Report < IMGT/HLA < IPD < EMBL-EBI". https://www.ebi.ac.uk/cgi-bin/ipd/imgt/hla/get_allele.cgi?DQB1*0201. 
  9. "HLA-DQB1*0201 homozygosis predisposes to severe intestinal damage in celiac disease". Scand. J. Gastroenterol. 42 (1): 48–53. 2007. doi:10.1080/00365520600789859. PMID 17190762.  https://dx.doi.org/10.1080%2F00365520600789859
  10. "Intraepithelial lymphocytes". Current Biology 27 (15): R737–R739. August 2017. doi:10.1016/j.cub.2017.05.073. PMID 28787597.  https://dx.doi.org/10.1016%2Fj.cub.2017.05.073
  11. "Intestinal Intraepithelial Lymphocytes: Sentinels of the Mucosal Barrier". Trends in Immunology 39 (4): 264–275. April 2018. doi:10.1016/j.it.2017.11.003. PMID 29221933.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=8056148
  12. "Approach to patients with refractory coeliac disease". F1000Research 5: 2544. 2016. doi:10.12688/f1000research.9051.1. PMID 27803799.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5074352
  13. "Diagnosis and Treatment Patterns in Celiac Disease". Digestive Diseases and Sciences 64 (8): 2095–2106. March 2019. doi:10.1007/s10620-019-05528-3. PMID 30820708.  https://dx.doi.org/10.1007%2Fs10620-019-05528-3
  14. "CD4 T-cell cytokines synergize to induce proliferation of malignant and nonmalignant innate intraepithelial lymphocytes". Proceedings of the National Academy of Sciences of the United States of America 114 (6): E980–E989. February 2017. doi:10.1073/pnas.1620036114. PMID 28049849.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5307453
  15. "Novel findings from the Asian Lymphoma Study Group: focus on T and NK-cell lymphomas". International Journal of Hematology 107 (4): 413–419. April 2018. doi:10.1007/s12185-018-2406-6. PMID 29380182.  https://dx.doi.org/10.1007%2Fs12185-018-2406-6
  16. "Recent advances in intestinal lymphomas". Histopathology 66 (1): 112–36. January 2015. doi:10.1111/his.12596. PMID 25639480.  https://dx.doi.org/10.1111%2Fhis.12596
  17. "Enteropathy-associated T cell lymphoma subtypes are characterized by loss of function of SETD2". The Journal of Experimental Medicine 214 (5): 1371–1386. May 2017. doi:10.1084/jem.20160894. PMID 28424246.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=5413324
  18. "Small Bowel Adenocarcinoma in a Patient with Coeliac Disease: Case Report and Review of the Literature". Case Reports in Gastroenterology 4 (3): 416–420. October 2010. doi:10.1159/000313547. PMID 21060711.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=2975010
  19. "Small bowel adenocarcinoma as a complication of celiac disease: clinical and diagnostic features". BMC Gastroenterology 19 (1): 45. March 2019. doi:10.1186/s12876-019-0964-6. PMID 30917787.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=6437995
  20. "A Challenging Diagnosis of Jejunal Adenocarcinoma in a Celiac Patient: Case Report and Systematic Review of the Literature". Journal of Gastrointestinal and Liver Diseases 26 (4): 411–415. December 2017. doi:10.15403/jgld.2014.1121.264.zet. PMID 29253057.  https://dx.doi.org/10.15403%2Fjgld.2014.1121.264.zet
  21. "Celiac disease". The New England Journal of Medicine 357 (17): 1731–43. October 2007. doi:10.1056/NEJMra071600. PMID 17960014.  https://dx.doi.org/10.1056%2FNEJMra071600
  22. "Small bowel adenocarcinoma complicating coeliac disease: a report of three cases and the literature review". Case Reports in Oncological Medicine 2012: 935183. 2012. doi:10.1155/2012/935183. PMID 23243535.  http://www.pubmedcentral.nih.gov/articlerender.fcgi?tool=pmcentrez&artid=3517832
  23. "Peripheral T-cell lymphoma, not otherwise specified". Blood 129 (9): 1103–1112. March 2017. doi:10.1182/blood-2016-08-692566. PMID 28115372.  https://dx.doi.org/10.1182%2Fblood-2016-08-692566
  24. "The biology and management of systemic anaplastic large cell lymphoma". Blood 126 (1): 17–25. July 2015. doi:10.1182/blood-2014-10-567461. PMID 25869285.  https://dx.doi.org/10.1182%2Fblood-2014-10-567461
  25. "Extranodal NK/T-cell lymphoma: Updates in biology and management strategies". Best Practice & Research. Clinical Haematology 31 (3): 315–321. September 2018. doi:10.1016/j.beha.2018.07.002. PMID 30213402.  https://dx.doi.org/10.1016%2Fj.beha.2018.07.002
  26. "Epstein-Barr Virus and Cancer". Annual Review of Pathology 14: 29–53. 2019. doi:10.1146/annurev-pathmechdis-012418-013023. PMID 30125149.  https://dx.doi.org/10.1146%2Fannurev-pathmechdis-012418-013023
  27. "Indolent T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: a review and update". Hematological Oncology 35 (1): 3–16. March 2017. doi:10.1002/hon.2317. PMID 27353398.  https://dx.doi.org/10.1002%2Fhon.2317
  28. "New insights in the pathogenesis of T-cell lymphomas". Current Opinion in Oncology 30 (5): 277–284. September 2018. doi:10.1097/CCO.0000000000000474. PMID 30028743. https://serval.unil.ch/resource/serval:BIB_3FF9FC10B4B3.P001/REF.pdf. 
  29. Jantunen, E; Boumendil, A; Finel, H; Luan, J. J.; Johnson, P; Rambaldi, A; Haynes, A; Duchosal, M. A. et al. (2013). "Autologous stem cell transplantation for enteropathy-associated T-cell lymphoma: A retrospective study by the EBMT". Blood 121 (13): 2529–32. doi:10.1182/blood-2012-11-466839. PMID 23361910.  https://dx.doi.org/10.1182%2Fblood-2012-11-466839
  30. "Home Page". http://www.institutimagine.org/en/imagine-institute.html. 
  31. "Phase 2 Study of Brentuximab Vedotin Associated with CHP Followed by Consolidation with High-dose Therapy / Autologous Stem-cell Transplantation as Frontline Treatment of Patients with Enteropathy-associated T-cell Lymphoma Type 1". 9 February 2021. https://clinicaltrials.gov/ct2/show/NCT03217643?cond=Enteropathy-associated+T-cell+lymphoma&rank=1. 
  32. "T Cells Expressing a Fully-Human Anti-CD30 Chimeric Antigen Receptor for Treating CD30-Expressing Lymphomas - Tabular View - ClinicalTrials.gov". https://clinicaltrials.gov/ct2/show/record/NCT03049449. 
  33. "Nivolumab in Treating Patients With Relapsed or Refractory Peripheral T-cell Lymphoma - Tabular View - ClinicalTrials.gov". https://clinicaltrials.gov/ct2/show/record/NCT03075553. 
  34. "A Phase 2a, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of AMG 714 in Adult Patients with Type II Refractory Celiac Disease, an in Situ Small Bowel T Cell Lymphoma". 11 December 2019. https://clinicaltrials.gov/ct2/show/NCT02633020?cond=refractory+celiac+disease&rank=1. 
  35. "A Phase 2a, Randomized, Double-Blind, Placebo-Controlled, Parallel-Group Study to Evaluate the Efficacy and Safety of AMG 714 in Adult Patients with Type II Refractory Celiac Disease, an in Situ Small Bowel T Cell Lymphoma". 11 December 2019. https://clinicaltrials.gov/ct2/show/NCT02633020?term=NCT03439475+%5BExpandedAccessNCTId%5D&rank=1. 
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