Pathogenesis of Enteropathy-Associated T Cell Lymphoma.

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. The immune microenvironment of the small intestinal mucosa in celiac disease fosters the development of EATL, often in a multistep pathway.

journal_name

Curr Hematol Malig Rep

authors

Chander U,Leeman-Neill RJ,Bhagat G

doi

10.1007/s11899-018-0459-5

subject

Has Abstract

pub_date

2018-08-01 00:00:00

pages

308-317

issue

4

eissn

1558-8211

issn

1558-822X

pii

10.1007/s11899-018-0459-5

journal_volume

13

pub_type

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