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Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy

Abstract: Primary cutaneous T-cell lymphomas (CTCLs) constitute a heterogeneous group of diseases that affect the skin. Mycosis fungoides (MF) and Sézary syndrome (SS) account for the majority of these lesions and have recently been the focus of extensive translational research. This review describes and discusses the main pathobiological manifestations of MF/SS, the molecular and clinical features currently used for diagnosis and staging, and the different therapies already approved or under development. Furthermore, we highlight and discuss the main findings illuminating key molecular mechanisms that can act as drivers for the development and progression of MF/SS. These seem to make up an orchestrated constellation of genomic and environmental alterations generated around deregulated T-cell receptor (TCR)/phospholipase C, gamma 1, (PLCG1) and Janus kinase/ signal transducer and activator of transcription (JAK/STAT) activities that do indeed provide us with novel opportunities for diagnosis and therapy.

 Fuente: Cancers (Basel) . 2021 Apr 16;13(8):1931

Editorial: MDPI

 Año de publicación: 2021

Nº de páginas: 24

Tipo de publicación: Artículo de Revista

 DOI: 10.3390/cancers13081931

ISSN: 2072-6694

Url de la publicación: https://doi.org/10.3390/cancers13081931

Autores/as

NURIA GARCIA DIAZ

PIRIS, MIGUEL ÁNGEL

ORTIZ-ROMERO, PABLO LUIS