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Indolent T-Cell Lymphoproliferative Disorder of the Gastrointestinal Tract

MedGen UID:
1611878
•Concept ID:
C4528218
•
Neoplastic Process
Synonym: Indolent T-cell lymphoproliferative disorder of gastrointestinal tract
SNOMED CT: Indolent T-cell lymphoproliferative disorder of gastrointestinal tract (783565007); Indolent T-cell lymphoproliferative disorder of gastrointestinal tract (788565009)

Definition

A clonal T-cell lymphoproliferative disorder that can involve the mucosa in all sites of the gastrointestinal tract, but is most common in the small intestine and colon. The lymphoid cells infiltrate the lamina propria but usually do not show invasion of the epithelium. The clinical course is indolent, but most patients do not respond to conventional chemotherapy. A subset of cases progress to a higher-grade T-cell lymphoma with spread beyond the gastrointestinal tract. (WHO 2017) [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVIndolent T-Cell Lymphoproliferative Disorder of the Gastrointestinal Tract

Professional guidelines

PubMed

American Registry of Pathology Expert Opinions: Recommendations for the diagnostic workup of mature T cell neoplasms.
Vega F, Amador C, Chadburn A, Feldman AL, Hsi ED, Wang W, Medeiros LJ
Ann Diagn Pathol 2020 Dec;49:151623. Epub 2020 Sep 13 doi: 10.1016/j.anndiagpath.2020.151623. PMID: 32947231
Biology and management of mantle cell lymphoma.
Leonard JP, Schattner EJ, Coleman M
Curr Opin Oncol 2001 Sep;13(5):342-7. doi: 10.1097/00001622-200109000-00005. PMID: 11555710

Recent clinical studies

Etiology

Indolent T-cell lymphoproliferative disorder of gastrointestinal tract with unusual clinical courses: report of 6 cases and literature review.
Fan W, Niu L, He H, Yuan J, Yuan F, Shi X, Wang Y, Chen M, Huang M, Zhou F, Xu J, Chen Q
Virchows Arch 2023 Apr;482(4):729-743. Epub 2022 Dec 6 doi: 10.1007/s00428-022-03467-5. PMID: 36472661
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

Diagnosis

Indolent T-cell lymphoproliferative disorder of gastrointestinal tract with unusual clinical courses: report of 6 cases and literature review.
Fan W, Niu L, He H, Yuan J, Yuan F, Shi X, Wang Y, Chen M, Huang M, Zhou F, Xu J, Chen Q
Virchows Arch 2023 Apr;482(4):729-743. Epub 2022 Dec 6 doi: 10.1007/s00428-022-03467-5. PMID: 36472661
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
Indolent T cell lymphoproliferative disorder with villous atrophy in small intestine diagnosed by single-balloon enteroscopy.
Nagaishi T, Yamada D, Suzuki K, Fukuyo R, Saito E, Fukuda M, Watabe T, Tsugawa N, Takeuchi K, Yamamoto K, Arai A, Ohtsuka K, Watanabe M
Clin J Gastroenterol 2019 Oct;12(5):434-440. Epub 2019 Apr 9 doi: 10.1007/s12328-019-00971-1. PMID: 30968266Free PMC Article

Prognosis

Indolent T-cell lymphoproliferative disorder of gastrointestinal tract with unusual clinical courses: report of 6 cases and literature review.
Fan W, Niu L, He H, Yuan J, Yuan F, Shi X, Wang Y, Chen M, Huang M, Zhou F, Xu J, Chen Q
Virchows Arch 2023 Apr;482(4):729-743. Epub 2022 Dec 6 doi: 10.1007/s00428-022-03467-5. PMID: 36472661
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

Clinical prediction guides

Indolent T cell lymphoproliferative disorder with villous atrophy in small intestine diagnosed by single-balloon enteroscopy.
Nagaishi T, Yamada D, Suzuki K, Fukuyo R, Saito E, Fukuda M, Watabe T, Tsugawa N, Takeuchi K, Yamamoto K, Arai A, Ohtsuka K, Watanabe M
Clin J Gastroenterol 2019 Oct;12(5):434-440. Epub 2019 Apr 9 doi: 10.1007/s12328-019-00971-1. PMID: 30968266Free PMC Article

Supplemental Content

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