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Intestinal T-Cell Lymphoma

MedGen UID:: 1656655
•Concept ID:: C4721452
•: Neoplastic Process

Synonym:	Intestinal T-cell lymphoma
SNOMED CT:	Intestinal T-cell lymphoma (103686008)

Definition

A group of mature T-cell and NK-cell non-Hodgkin lymphomas that includes enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, and intestinal T-cell lymphoma, not otherwise specified. Most of these lymphomas arise from the small intestine and a minority from the large intestine or stomach. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVIntestinal T-Cell Lymphoma

Hematologic neoplasm
- Lymphoma
  - Non-Hodgkin lymphoma
    - Non-Hodgkin Lymphoma by Clinical Course
      - Aggressive Non-Hodgkin Lymphoma
        Aggressive T-Cell Non-Hodgkin Lymphoma
        Intestinal T-Cell Lymphoma
        Enteropathy-associated T-cell lymphoma
        Enteropathy-Associated T-Cell Lymphoma by Ann Arbor Stage
        Ann Arbor Stage I Enteropathy-Associated T-Cell Lymphoma
        Ann Arbor Stage II Enteropathy-Associated T-Cell Lymphoma
        Ann Arbor Stage III Enteropathy-Associated T-Cell Lymphoma
        Ann Arbor Stage IV Enteropathy-Associated T-Cell Lymphoma
        Recurrent Enteropathy-Associated T-Cell Lymphoma
        Refractory Enteropathy-Associated T-Cell Lymphoma
        Small intestinal enteropathy-associated T-cell lymphoma
        Intestinal T-Cell Lymphoma, Not Otherwise Specified
        Monomorphic epitheliotropic intestinal T-cell lymphoma
        Recurrent Monomorphic Epitheliotropic Intestinal T-cell Lymphoma
        Refractory Monomorphic Epitheliotropic Intestinal T-cell Lymphoma
        Small Intestinal Monomorphic Epitheliotropic T-Cell Lymphoma

Professional guidelines

PubMed

Current and upcoming treatment approaches to uncommon subtypes of PTCL (EATL, MEITL, SPTCL, and HSTCL).

Marchi E, Craig JW, Kalac M
Blood 2024 Oct 31;144(18):1898-1909. doi: 10.1182/blood.2023021788. PMID: 38657272

ACVIM consensus statement guidelines on diagnosing and distinguishing low-grade neoplastic from inflammatory lymphocytic chronic enteropathies in cats.

Marsilio S, Freiche V, Johnson E, Leo C, Langerak AW, Peters I, Ackermann MR
J Vet Intern Med 2023 May-Jun;37(3):794-816. Epub 2023 May 2 doi: 10.1111/jvim.16690. PMID: 37130034 Free PMC Article

Management of celiac disease in daily clinical practice.

Elli L, Ferretti F, Orlando S, Vecchi M, Monguzzi E, Roncoroni L, Schuppan D
Eur J Intern Med 2019 Mar;61:15-24. Epub 2018 Dec 5 doi: 10.1016/j.ejim.2018.11.012. PMID: 30528262

See all (8)

Recent clinical studies

Etiology

ACVIM consensus statement guidelines on diagnosing and distinguishing low-grade neoplastic from inflammatory lymphocytic chronic enteropathies in cats.

Marsilio S, Freiche V, Johnson E, Leo C, Langerak AW, Peters I, Ackermann MR
J Vet Intern Med 2023 May-Jun;37(3):794-816. Epub 2023 May 2 doi: 10.1111/jvim.16690. PMID: 37130034 Free PMC Article

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: 35708139 Free PMC Article

Intestinal T-cell lymphoma presenting as colonic perforation in the setting of ulcerative colitis: a case report.

Mourad AP, De Robles MS, Winn RD
Clin J Gastroenterol 2021 Feb;14(1):176-180. Epub 2020 Nov 10 doi: 10.1007/s12328-020-01279-1. PMID: 33174156

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.

Abdallah H, Leffler D, Dennis M, Kelly CP
Curr Gastroenterol Rep 2007 Oct;9(5):401-5. doi: 10.1007/s11894-007-0049-5. PMID: 17991341

See all (53)

Diagnosis

ACVIM consensus statement guidelines on diagnosing and distinguishing low-grade neoplastic from inflammatory lymphocytic chronic enteropathies in cats.

Marsilio S, Freiche V, Johnson E, Leo C, Langerak AW, Peters I, Ackermann MR
J Vet Intern Med 2023 May-Jun;37(3):794-816. Epub 2023 May 2 doi: 10.1111/jvim.16690. PMID: 37130034 Free PMC Article

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma (MEITL) Within a Meckel's Diverticulum.

Fontaine Kent L, Rodgers DL, McGhee JA, Patel VG
Am Surg 2021 Jan;87(1):101-102. Epub 2020 Aug 22 doi: 10.1177/0003134820943563. PMID: 32830541

Monomorphic Epitheliotropic Intestinal T-Cell Lymphoma: A Rare Case of Diffuse Ulcers in the Intestine.

Liu C, Feng L, Sun X
Clin Gastroenterol Hepatol 2021 Dec;19(12):e121-e122. Epub 2020 Jul 12 doi: 10.1016/j.cgh.2020.07.011. PMID: 32668339

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

Monomorphic epitheliotropic intestinal T cell lymphoma presenting in weblike feature.

Noh CK, Roh J, Lee KJ
Gastrointest Endosc 2019 Apr;89(4):891-892. Epub 2018 Dec 18 doi: 10.1016/j.gie.2018.12.009. PMID: 30576648

See all (93)

Therapy

Peripheral T-cell lymphoma with gastrointestinal involvement and indolent T-lymphoproliferative disorders of the gastrointestinal tract.

Kohri M, Tsukasaki K, Akuzawa Y, Tanae K, Takahashi N, Saeki T, Okamura D, Ishikawa M, Maeda T, Kawai N, Matsuda A, Arai E, Arai S, Asou N
Leuk Res 2020 Apr;91:106336. Epub 2020 Feb 29 doi: 10.1016/j.leukres.2020.106336. PMID: 32151888

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

Intestinal T-cell lymphomas: a retrospective analysis of 68 cases in China.

Sun ZH, Zhou HM, Song GX, Zhou ZX, Bai L
World J Gastroenterol 2014 Jan 7;20(1):296-302. doi: 10.3748/wjg.v20.i1.296. PMID: 24415885 Free PMC Article

Refractory celiac disease.

Abdallah H, Leffler D, Dennis M, Kelly CP
Curr Gastroenterol Rep 2007 Oct;9(5):401-5. doi: 10.1007/s11894-007-0049-5. PMID: 17991341

Treatment of T-cell non-Hodgkin's lymphoma.

Evens AM, Gartenhaus RB
Curr Treat Options Oncol 2004 Aug;5(4):289-303. doi: 10.1007/s11864-004-0020-8. PMID: 15233906

See all (25)

Prognosis

Monomorphic Epitheliotropic Intestinal T Cell Lymphoma of the Appendix: a Case Report and Review of Literature.

Soardo G, Castaldo V, Donnini D, Uzzau A, Pizzolitto S, Sechi LA
J Gastrointest Cancer 2020 Jun;51(2):688-694. doi: 10.1007/s12029-020-00363-6. PMID: 31970655

T- and NK-cell lymphoproliferative disorders of the gastrointestinal tract: review and update.

van Vliet C, Spagnolo DV
Pathology 2020 Jan;52(1):128-141. Epub 2019 Nov 11 doi: 10.1016/j.pathol.2019.10.001. PMID: 31727264

Monomorphic Epitheliotropic Intestinal T cell Lymphoma: a Rare Cause of Chronic Diarrhea.

Samuel R, Krill T, Merwat S
J Gastrointest Cancer 2019 Dec;50(4):1051-1054. doi: 10.1007/s12029-019-00210-3. PMID: 30761475

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

Refractory celiac disease.

Abdallah H, Leffler D, Dennis M, Kelly CP
Curr Gastroenterol Rep 2007 Oct;9(5):401-5. doi: 10.1007/s11894-007-0049-5. PMID: 17991341

See all (59)

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: 38520011 Free PMC Article

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: 33087157 Free PMC Article

Peripheral T-cell lymphoma with gastrointestinal involvement and indolent T-lymphoproliferative disorders of the gastrointestinal tract.

Intestinal T-cell lymphomas: a retrospective analysis of 68 cases in China.

Sun ZH, Zhou HM, Song GX, Zhou ZX, Bai L
World J Gastroenterol 2014 Jan 7;20(1):296-302. doi: 10.3748/wjg.v20.i1.296. PMID: 24415885 Free PMC Article

Pathology of intestinal lymphomas.

Foss HD, Stein H
Recent Results Cancer Res 2000;156:33-41. doi: 10.1007/978-3-642-57054-4_5. PMID: 10802861

See all (19)