Pathogenesis of Peripheral T Cell Lymphoma.

TitlePathogenesis of Peripheral T Cell Lymphoma.
Publication TypeJournal Article
Year of Publication2018
AuthorsPizzi M, Margolskee E, Inghirami G
JournalAnnu Rev Pathol
Volume13
Pagination293-320
Date Published2018 01 24
ISSN1553-4014
KeywordsHumans, Lymphoma, T-Cell, Peripheral
Abstract

Peripheral T cell lymphomas (PTCLs) are highly heterogeneous tumors, displaying distinct clinical and biological features. The pathogenesis and normal counterpart of such entities have been elusive for decades. Recent studies have, however, disclosed key mechanisms of peripheral T cell transformation, including (a) the deregulation of signaling pathways controlling T cell development, differentiation, and maturation; (b) the remodeling of the peritumor microenvironment; and (c) the virus-mediated rewiring of T cell biology. Uncovering the molecular mechanisms of T cell transformation will help elucidate the peculiar clinical and pathological features of each PTCL entity and will lead to the characterization of novel antitumor therapies. These therapies will combine conventional and new-generation compounds with immune-modulating agents to ablate both the neoplastic cells and the tumor-supporting microenvironment. This review addresses the pathogenic mechanisms of PTCLs, with special attention paid to novel therapeutic strategies for the clinical management of such aggressive tumors.

DOI10.1146/annurev-pathol-020117-043821
Alternate JournalAnnu Rev Pathol
PubMed ID29414251
Related Faculty: 
Giorgio Inghirami, M.D.

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