title: Indolent T-cell lymphoproliferative disease of the gastrointestinal tract.
abstract: Primary gastrointestinal (GI) T-cell lymphoma is an infrequent and aggressive disease. However, rare indolent clonal T-cell proliferations in the GI tract have been described. We report 10 cases of GI involvement by an indolent T-cell lymphoproliferative disease, including 6 men and 4 women with a median age of 48 years (range, 15-77 years). Presenting symptoms included abdominal pain, diarrhea, vomiting, food intolerance, and dyspepsia. The lesions involved oral cavity, esophagus, stomach, small intestine, and colon. The infiltrates were dense, but nondestructive, and composed of small, mature-appearing lymphoid cells. Eight cases were CD4(-)/CD8(+), 1 was CD4(+)/CD8(-), and another was CD4(-)/CD8(-). T-cell receptor-γ chain gene rearrangement identified a clonal population in all 10 cases. There was no evidence of STAT3 SH2 domain mutation or activation. Six patients received chemotherapy because of an initial diagnosis of peripheral T-cell lymphoma, with little or no response, whereas the other 4 were followed without therapy. After a median follow-up of 38 months (range, 9-175 months), 9 patients were alive with persistent disease and 1 was free of disease. We propose the name "indolent T-LPD of the GI tract" for these lesions that can easily be mistaken for intestinal peripheral T-cell lymphoma, and lead to aggressive therapy.
authors.affiliation: Department of Pathology, University of Manitoba, Winnipeg, MB, Canada;
authors.forename: Anamarija M
authors.initials: AM
authors.lastname: Perry
authors.forename: Roger A
authors.initials: RA
authors.lastname: Warnke
authors.forename: Qinglong
authors.initials: Q
authors.lastname: Hu
authors.forename: Philippe
authors.initials: P
authors.lastname: Gaulard
authors.forename: Christiane
authors.initials: C
authors.lastname: Copie-Bergman
authors.forename: Serhan
authors.initials: S
authors.lastname: Alkan
authors.forename: Huan-You
authors.initials: HY
authors.lastname: Wang
authors.forename: Jason X
authors.initials: JX
authors.lastname: Cheng
authors.forename: Chris M
authors.initials: CM
authors.lastname: Bacon
authors.forename: Jan
authors.initials: J
authors.lastname: Delabie
authors.forename: Erik
authors.initials: E
authors.lastname: Ranheim
authors.forename: Can
authors.initials: C
authors.lastname: Kucuk
authors.forename: Xiaozhou
authors.initials: X
authors.lastname: Hu
authors.forename: Dennis D
authors.initials: DD
authors.lastname: Weisenburger
authors.forename: Elaine S
authors.initials: ES
authors.lastname: Jaffe
authors.forename: Wing C
authors.initials: WC
authors.lastname: Chan
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000378:metabolism; D003937:Diagnosis, Differential; D058527:Enteropathy-Associated T-Cell Lymphoma / Q000276:immunology / Q000473:pathology; D005260:Female; D005767:Gastrointestinal Diseases / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D005770:Gastrointestinal Neoplasms / Q000276:immunology / Q000473:pathology; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D006801:Humans; D016411:Lymphoma, T-Cell, Peripheral / Q000276:immunology / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009154:Mutation; D050796:STAT3 Transcription Factor / Q000235:genetics / Q000378:metabolism; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*; D009626:Terminology as Topic
chemical_list: D015703:Antigens, CD; D050796:STAT3 Transcription Factor; C494086:STAT3 protein, human