Anais Brasileiros de Dermatologia (Aug 2021)

Mycosis fungoides and Sézary syndrome: focus on the current treatment scenario

  • José Antonio Sanches,
  • Jade Cury-Martins,
  • Rodrigo Martins Abreu,
  • Denis Miyashiro,
  • Juliana Pereira

DOI
https://doi.org/10.1016/j.abd.2020.12.007
Journal volume & issue
Vol. 96, no. 4
pp. 458 – 471

Abstract

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Abstract Cutaneous T-cell lymphomas are a heterogeneous group of lymphoproliferative disorders, characterized by infiltration of the skin by mature malignant T cells. Mycosis fungoides is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases. Mycosis fungoides in the early-stage is generally an indolent disease, progressing slowly from some patches or plaques to more widespread skin involvement. However, 20% to 25% of patients progress to advanced stages, with the development of skin tumors, extracutaneous spread and poor prognosis. Treatment modalities can be divided into two groups: skin-directed therapies and systemic therapies. Therapies targeting the skin include topical agents, phototherapy and radiotherapy. Systemic therapies include biological response modifiers, immunotherapies and chemotherapeutic agents. For early-stage mycosis fungoides, skin-directed therapies are preferred, to control the disease, improve symptoms and quality of life. When refractory or in advanced-stage disease, systemic treatment is necessary. In this article, the authors present a compilation of current treatment options for mycosis fungoides and Sézary syndrome.

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