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Enteropathy-associated T-cell lymphoma(EATCL)

MedGen UID:
96832
Concept ID:
C0456889
Neoplastic Process
Synonyms: EATCL; High-grade pleomorphic peripheral T-cell lymphoma
SNOMED CT: Enteropathy type (intestinal) T-cell lymphoma (277654008); Enteropathy-type T-cell lymphoma (277654008); Enteropathy-associated T-cell lymphoma (277654008); EACTL - Enteropathy-associated T-cell lymphoma (277654008); EATL - enteropathy-associated T-cell lymphoma (1222550009); Enteropathy-associated T-cell lymphoma (1222550009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019473
Orphanet: ORPHA86880

Definition

A rare T-cell non-Hodgkin lymphoma characterized by a neoplasm of intraepithelial T-cells mostly occurring in the jejunum or ileum in patients with celiac disease. The lesion may be multifocal and form ulcerating nodules, plaques, strictures, or an exophytic mass. The mesentery and mesenteric lymph nodes are commonly involved. Patients typically present with abdominal pain, malabsorption or diarrhea, anorexia, weight loss, fatigue, nausea, vomiting, and sometimes intestinal perforation or hemorrhage. Prognosis is generally poor. [from ORDO]

Professional guidelines

PubMed

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
Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Al-Toma A, Volta U, Auricchio R, Castillejo G, Sanders DS, Cellier C, Mulder CJ, Lundin KEA
United European Gastroenterol J 2019 Jun;7(5):583-613. Epub 2019 Apr 13 doi: 10.1177/2050640619844125. PMID: 31210940Free PMC Article

Recent clinical studies

Etiology

Veloza L, Cavalieri D, Missiaglia E, Ledoux-Pilon A, Bisig B, Pereira B, Bonnet C, Poullot E, Quintanilla-Martinez L, Dubois R, Llamas-Gutierrez F, Bossard C, De Wind R, Drieux F, Fontaine J, Parrens M, Sandrini J, Fataccioli V, Delfau-Larue MH, Daniel A, Lhomme F, Clément-Filliatre L, Lemonnier F, Cairoli A, Morel P, Glaisner S, Joly B, El Yamani A, Laribi K, Bachy E, Siebert R, Vallois D, Gaulard P, Tournilhac O, De Leval L
Haematologica 2023 Jan 1;108(1):181-195. doi: 10.3324/haematol.2022.281226. PMID: 35708139Free PMC Article
Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Al Somali Z, Hamadani M, Kharfan-Dabaja M, Sureda A, El Fakih R, Aljurf M
Curr Hematol Malig Rep 2021 Apr;16(2):140-147. Epub 2021 May 19 doi: 10.1007/s11899-021-00634-4. PMID: 34009525
Al-Toma A, Volta U, Auricchio R, Castillejo G, Sanders DS, Cellier C, Mulder CJ, Lundin KEA
United European Gastroenterol J 2019 Jun;7(5):583-613. Epub 2019 Apr 13 doi: 10.1177/2050640619844125. PMID: 31210940Free PMC Article
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

Diagnosis

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
Horwitz SM, Ansell S, Ai WZ, Barnes J, Barta SK, Brammer J, Clemens MW, Dogan A, Foss F, Ghione P, Goodman AM, Guitart J, Halwani A, Haverkos BM, Hoppe RT, Jacobsen E, Jagadeesh D, Jones A, Kallam A, Kim YH, Kumar K, Mehta-Shah N, Olsen EA, Rajguru SA, Rozati S, Said J, Shaver A, Shea L, Shinohara MM, Sokol L, Torres-Cabala C, Wilcox R, Wu P, Zain J, Dwyer M, Sundar H
J Natl Compr Canc Netw 2022 Mar;20(3):285-308. doi: 10.6004/jnccn.2022.0015. PMID: 35276674
Al Somali Z, Hamadani M, Kharfan-Dabaja M, Sureda A, El Fakih R, Aljurf M
Curr Hematol Malig Rep 2021 Apr;16(2):140-147. Epub 2021 May 19 doi: 10.1007/s11899-021-00634-4. PMID: 34009525
Al-Toma A, Volta U, Auricchio R, Castillejo G, Sanders DS, Cellier C, Mulder CJ, Lundin KEA
United European Gastroenterol J 2019 Jun;7(5):583-613. Epub 2019 Apr 13 doi: 10.1177/2050640619844125. PMID: 31210940Free PMC Article
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

Therapy

Hujoel IA, Murray JA
Curr Gastroenterol Rep 2020 Mar 17;22(4):18. doi: 10.1007/s11894-020-0756-8. PMID: 32185560
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
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603
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
Green PH, Jabri B
Lancet 2003 Aug 2;362(9381):383-91. doi: 10.1016/S0140-6736(03)14027-5. PMID: 12907013

Prognosis

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
Al Somali Z, Hamadani M, Kharfan-Dabaja M, Sureda A, El Fakih R, Aljurf M
Curr Hematol Malig Rep 2021 Apr;16(2):140-147. Epub 2021 May 19 doi: 10.1007/s11899-021-00634-4. PMID: 34009525
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
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603
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

Ishibashi H, Nimura S, Hirai F, Harada N, Iwasaki H, Kawauchi S, Oshiro Y, Matsuyama A, Nakamura S, Takamatsu Y, Yonemasu H, Shimokama T, Takeshita M
Diagn Pathol 2020 Oct 21;15(1):128. doi: 10.1186/s13000-020-01044-5. PMID: 33087157Free PMC Article
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
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
de Baaij LR, Berkhof J, van de Water JM, Sieniawski MK, Radersma M, Verbeek WH, Visser OJ, Oudejans JJ, Meijer CJ, Mulder CJ, Lennard AL, Cillessen SA
Clin Cancer Res 2015 Jul 1;21(13):3013-9. Epub 2015 Mar 16 doi: 10.1158/1078-0432.CCR-14-2195. PMID: 25779949
Swerdlow SH, Jaffe ES, Brousset P, Chan JK, de Leval L, Gaulard P, Harris NL, Pileri S, Weiss LM; International Lymphoma Study Group
Am J Surg Pathol 2014 Oct;38(10):e60-71. doi: 10.1097/PAS.0000000000000295. PMID: 25025449Free PMC Article

Recent systematic reviews

Gromny I, Neubauer K
Int J Environ Res Public Health 2023 Jan 14;20(2) doi: 10.3390/ijerph20021565. PMID: 36674320Free PMC Article
Rowinski SA, Christensen E
Dan Med J 2016 Dec;63(12) PMID: 27910801
Labidi A, Serghini M, Karoui S, Boubaker J, Filali A
Tunis Med 2013 Aug-Sep;91(8-9):493-8. PMID: 24227505

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