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García-Díaz N, Piris MÁ, Ortiz-Romero PL, et al. Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy. Cancers (Basel). 2021;13(8)doi: 10.3390/cancers13081931.
García-Díaz, N., Piris, M. �. �., Ortiz-Romero, P. L., & Vaqué, J. P. (2021). Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy. Cancers, 13(8), . https://doi.org/10.3390/cancers13081931
García-Díaz, Nuria, et al. "Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy." Cancers vol. 13,8 (2021). doi: https://doi.org/10.3390/cancers13081931
García-Díaz N, Piris MÁ, Ortiz-Romero PL, Vaqué JP. Mycosis Fungoides and Sézary Syndrome: An Integrative Review of the Pathophysiology, Molecular Drivers, and Targeted Therapy. Cancers (Basel). 2021 Apr 16;13(8). doi: 10.3390/cancers13081931. PMID: 33923722; PMCID: PMC8074086.
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Cancers (Basel). 2021 Apr 16;13(8). doi: 10.3390/cancers13081931.

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

Cancers

Nuria García-Díaz, Miguel Ángel Piris, Pablo Luis Ortiz-Romero, José Pedro Vaqué

Affiliations

  1. Molecular Biology Department, Universidad de Cantabria-Instituto de Investigación Marqués de Valdecilla, IDIVAL, 39011 Santander, Spain.
  2. Department of Pathology, Fundación Jiménez Díaz, CIBERONC, 28040 Madrid, Spain.
  3. Department of Dermatology, Hospital 12 de Octubre, Institute i+12, CIBERONC, Medical School, University Complutense, 28041 Madrid, Spain.

PMID: 33923722 PMCID: PMC8074086 DOI: 10.3390/cancers13081931

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.

Keywords: CTCL; Sézary syndrome; diagnosis; molecular drivers; mycosis fungoides; therapy

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