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Current Hematologic Malignancy Reports
Erschienen in: Current Hematologic Malignancy Reports 4/2018

15.06.2018 | T-Cell and Other Lymphoproliferative Malignancies (J Zain, Section Editor)

Pathogenesis of Enteropathy-Associated T Cell Lymphoma

verfasst von: Udit Chander, Rebecca J. Leeman-Neill, Govind Bhagat

Erschienen in: Current Hematologic Malignancy Reports | Ausgabe 4/2018

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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.
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Metadaten
Titel
Pathogenesis of Enteropathy-Associated T Cell Lymphoma
verfasst von
Udit Chander
Rebecca J. Leeman-Neill
Govind Bhagat
Publikationsdatum
15.06.2018
Verlag
Springer US
Erschienen in
Current Hematologic Malignancy Reports / Ausgabe 4/2018
Print ISSN: 1558-8211
Elektronische ISSN: 1558-822X
DOI
https://doi.org/10.1007/s11899-018-0459-5

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