Cancers, Vol. 13, Pages 1931: Mycosis Fungoides and S ézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy
Cancers, Vol. 13, Pages 1931: Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy
Cancers doi: 10.3390/cancers13081931
Authors:
Nuria García-Díaz
Miguel Ángel Piris
Pablo Luis Ortiz‐Romero
José Pedro Vaqué
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.
Source: Cancers - Category: Cancer & Oncology Authors: Nuria Garc ía-Díaz Miguel Ángel Piris Pablo Luis Ortiz ‐Romero Jos é Pedro Vaqué Tags: Review Source Type: research
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