Get 20M+ Full-Text Papers For Less Than $1.50/day. Start a 14-Day Trial for You or Your Team.

Learn More →

Pathogenesis of Enteropathy-Associated T Cell Lymphoma

Pathogenesis of Enteropathy-Associated T Cell Lymphoma Purpose of Review To provide an update on the pathogenesis of enteropathy-associated T cell lymphoma (EATL) and its relationship with refractory celiac disease (RCD), in light of current knowledge of immune, genetic, and environmental factors that promote neoplastic transformation of intraepithelial lymphocytes (IELs). Recent Findings EATL frequently evolves from RCD type II (RCD II) but can occur “de novo” in individuals with celiac disease. Recurrent activating mutations in members of the JAK/STAT pathway have been recently described in EATL and RCD II, which suggests deregulation of cytokine signaling to be an early event in lymphomagenesis. Intraepithelial T cells are presumed to be the cell of origin of EATL (and RCD II). Recent in vitro molecular and phenotypic analyses and in vivo murine studies, however, suggest an origin of RCD II from innate IELs (NK/T cell precursors), which could also be the cell of origin of RCD II-derived EATL. Summary The immune microenvironment of the small intestinal mucosa in celiac disease fosters the development of EATL, often in a multistep pathway. . . . . . Keywords EATL Tcell lymphoma Celiac disease Refractory celiac disease Intestine Genetics Introduction lymphomas occurring in individuals with celiac disease and re- classification of EATL http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Hematologic Malignancy Reports Springer Journals

Pathogenesis of Enteropathy-Associated T Cell Lymphoma

Download PDF
10 pages

Loading next page...
 
/lp/springer-journals/pathogenesis-of-enteropathy-associated-t-cell-lymphoma-WWd8jHEGeU

References (112)

Publisher
Springer Journals
Copyright
Copyright © 2018 by Springer Science+Business Media, LLC, part of Springer Nature
Subject
Medicine & Public Health; Hematology; Oncology; Geriatrics/Gerontology
ISSN
1558-8211
eISSN
1558-822X
DOI
10.1007/s11899-018-0459-5
Publisher site
See Article on Publisher Site

Abstract

Purpose of Review To provide an update on the pathogenesis of enteropathy-associated T cell lymphoma (EATL) and its relationship with refractory celiac disease (RCD), in light of current knowledge of immune, genetic, and environmental factors that promote neoplastic transformation of intraepithelial lymphocytes (IELs). Recent Findings EATL frequently evolves from RCD type II (RCD II) but can occur “de novo” in individuals with celiac disease. Recurrent activating mutations in members of the JAK/STAT pathway have been recently described in EATL and RCD II, which suggests deregulation of cytokine signaling to be an early event in lymphomagenesis. Intraepithelial T cells are presumed to be the cell of origin of EATL (and RCD II). Recent in vitro molecular and phenotypic analyses and in vivo murine studies, however, suggest an origin of RCD II from innate IELs (NK/T cell precursors), which could also be the cell of origin of RCD II-derived EATL. Summary The immune microenvironment of the small intestinal mucosa in celiac disease fosters the development of EATL, often in a multistep pathway. . . . . . Keywords EATL Tcell lymphoma Celiac disease Refractory celiac disease Intestine Genetics Introduction lymphomas occurring in individuals with celiac disease and re- classification of EATL

Journal

Current Hematologic Malignancy Reports – Springer Journals

Published: Jun 15, 2018

There are no references for this article.