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Monomorphic epitheliotropic intestinal T-cell lymphoma

MedGen UID:
474158
•Concept ID:
C3272525
•
Neoplastic Process
Synonyms: Monomorphic CD56+ Intestinal T-Cell Lymphoma; Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma; monomorphic epitheliotropic intestinal T-cell lymphoma; Type II EATL
SNOMED CT: Monomorphic epitheliotropic intestinal T-cell lymphoma (787036009)
 
Monarch Initiative: MONDO:0958096

Definition

A mature T-cell and NK-cell non-Hodgkin lymphoma of intraepithelial T-lymphocytes. It usually arises from the small intestine. A minority of cases arise from the large intestine or the stomach. It is characterized by the presence of a monomorphic cellular infiltrate of small to medium-sized T-lymphocytes that are cytotoxic and express CD56. It is not associated with celiac disease. [from NCI]

Recent clinical studies

Etiology

Monomorphic epitheliotropic intestinal T-cell lymphoma comprises morphologic and genomic heterogeneity impacting outcome.
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
Targeted Next-generation Sequencing Reveals a Wide Morphologic and Immunophenotypic Spectrum of Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma.
Hang JF, Yuan CT, Chang KC, Wang RC, Chen BJ, Hsieh PP, Huang WT, Chuang WY, Chen TW, Yeh YC, Lin SY, Hsiao CH, Chou SC, Tseng CE, Pan ST, Chang SL, Chuang SS
Am J Surg Pathol 2022 Sep 1;46(9):1207-1218. Epub 2022 May 12 doi: 10.1097/PAS.0000000000001914. PMID: 35551151
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.
Fei F, Reddy V, Patel CR, Dhall D, Lee G, Meng-Jun X, Al Diffalha S
Ann Clin Lab Sci 2020 Nov;50(6):806-812. PMID: 33334797
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
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

Diagnosis

Monomorphic Epitheliotropic Intestinal T-cell Lymphoma of the Stomach: Two Case Reports and a Literature Review.
Lu S, Zhou G, Chen M, Liu W, Zhao S
Int J Surg Pathol 2021 Jun;29(4):410-419. Epub 2020 Aug 28 doi: 10.1177/1066896920953906. PMID: 32856508
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.
Fei F, Reddy V, Patel CR, Dhall D, Lee G, Meng-Jun X, Al Diffalha S
Ann Clin Lab Sci 2020 Nov;50(6):806-812. PMID: 33334797
Gastrointestinal T- and NK-cell lymphomas and indolent lymphoproliferative disorders.
Soderquist CR, Bhagat G
Semin Diagn Pathol 2020 Jan;37(1):11-23. Epub 2019 Aug 28 doi: 10.1053/j.semdp.2019.08.001. PMID: 31522873
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
Update on Gastrointestinal Lymphomas.
Weindorf SC, Smith LB, Owens SR
Arch Pathol Lab Med 2018 Nov;142(11):1347-1351. doi: 10.5858/arpa.2018-0275-RA. PMID: 30407861

Therapy

Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature.
Ozaka S, Inoue K, Okajima T, Tasaki T, Ariki S, Ono H, Ando T, Daa T, Murakami K
World J Gastroenterol 2021 Oct 14;27(38):6501-6510. doi: 10.3748/wjg.v27.i38.6501. PMID: 34720538Free PMC Article
Early central nervous system relapse of monomorphic epitheliotropic intestinal T-cell lymphoma after cord blood transplantation.
Nato Y, Miyazaki K, Imai H, Nakano E, Kageyama Y, Ino K, Fujieda A, Matsumoto T, Tawara I, Tanaka K, Yamaguchi M, Katayama N
Int J Hematol 2021 Jul;114(1):129-135. Epub 2021 Mar 1 doi: 10.1007/s12185-021-03107-9. PMID: 33646526
Multicenter retrospective analysis of the clinicopathologic features of monomorphic epitheliotropic intestinal T-cell lymphoma.
Yi JH, Lee GW, Do YR, Jung HR, Hong JY, Yoon DH, Suh C, Choi YS, Yi SY, Sohn BS, Kim BS, Oh SY, Park J, Jo JC, Lee SS, Oh YH, Kim SJ, Kim WS
Ann Hematol 2019 Nov;98(11):2541-2550. Epub 2019 Sep 6 doi: 10.1007/s00277-019-03791-y. PMID: 31493002
Enteropathy-Associated T-Cell Lymphoma.
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603
Multiple lesions of gastrointestinal tract invasion by monomorphic epitheliotropic intestinal T-cell lymphoma, accompanied by duodenal and intestinal enteropathy-like lesions and microscopic lymphocytic proctocolitis: a case series.
Ishibashi H, Nimura S, Kayashima Y, Takamatsu Y, Aoyagi K, Harada N, Kadowaki M, Kamio T, Sakisaka S, Takeshita M
Diagn Pathol 2016 Jul 25;11(1):66. doi: 10.1186/s13000-016-0519-x. PMID: 27457239Free PMC Article

Prognosis

Successful early diagnosis of monomorphic epitheliotropic intestinal T-cell lymphoma manifesting as chronic diarrhea and hypokalemia using video capsule endoscopy and double-balloon enteroscopy.
Naito T, Nosaka T, Takahashi K, Ofuji K, Matsuda H, Ohtani M, Hiramatsu K, Imamura Y, Yamauchi T, Nakamoto Y
Clin J Gastroenterol 2022 Oct;15(5):913-919. Epub 2022 Jul 30 doi: 10.1007/s12328-022-01676-8. PMID: 35907086
Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature.
Ozaka S, Inoue K, Okajima T, Tasaki T, Ariki S, Ono H, Ando T, Daa T, Murakami K
World J Gastroenterol 2021 Oct 14;27(38):6501-6510. doi: 10.3748/wjg.v27.i38.6501. PMID: 34720538Free PMC Article
Lymphomatous effusion of monomorphic epitheliotropic intestinal T-cell lymphoma is characterized by azurophilic granules and is a dismal sign: Report of two new cases with literature review.
Pan ST, Wang RC, Su YZ, Hsieh YC, Chuang SS
Diagn Cytopathol 2021 Jul;49(7):E247-E252. Epub 2021 Jan 2 doi: 10.1002/dc.24690. PMID: 33387400
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma: A Study of Four Cases and Review of Literature.
Fei F, Reddy V, Patel CR, Dhall D, Lee G, Meng-Jun X, Al Diffalha S
Ann Clin Lab Sci 2020 Nov;50(6):806-812. PMID: 33334797
Enteropathy-Associated T-Cell Lymphoma.
Ondrejka S, Jagadeesh D
Curr Hematol Malig Rep 2016 Dec;11(6):504-513. doi: 10.1007/s11899-016-0357-7. PMID: 27900603

Clinical prediction guides

Clinicopathological and molecular genetic alterations in monomorphic-epitheliotropic intestinal T-cell lymphoma of the small intestine.
Zhou B, Guo M, Li X, Duan T, Peng L, Hao H
Eur J Med Res 2024 Mar 23;29(1):191. doi: 10.1186/s40001-024-01797-5. PMID: 38520011Free PMC Article
Monomorphic epitheliotropic intestinal T-cell lymphoma presenting as melena with long-term survival: A case report and review of literature.
Ozaka S, Inoue K, Okajima T, Tasaki T, Ariki S, Ono H, Ando T, Daa T, Murakami K
World J Gastroenterol 2021 Oct 14;27(38):6501-6510. doi: 10.3748/wjg.v27.i38.6501. PMID: 34720538Free PMC Article
Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma With Secondary Cutaneous Involvement: A Diagnostic Challenge.
Olmos-Alpiste F, Vázquez I, Gallardo F, Sánchez-Gonzalez B, Colomo L, Pujol RM
Am J Dermatopathol 2021 Apr 1;43(4):300-304. doi: 10.1097/DAD.0000000000001855. PMID: 33264131
Monomorphic Epitheliotropic Intestinal T-cell Lymphoma of the Stomach: Two Case Reports and a Literature Review.
Lu S, Zhou G, Chen M, Liu W, Zhao S
Int J Surg Pathol 2021 Jun;29(4):410-419. Epub 2020 Aug 28 doi: 10.1177/1066896920953906. PMID: 32856508
Endoscopic and clinicopathological characteristics of colorectal T/NK cell lymphoma.
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

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