Mycosis Fungoides and Sézary Syndrome: An Update

Hematol Oncol Clin North Am. 2019 Feb;33(1):103-120. doi: 10.1016/j.hoc.2018.09.001.

Abstract

Cutaneous T-cell lymphomas are a heterogeneous collection of non-Hodgkin lymphomas that arise from skin-tropic memory T lymphocytes. Among them, mycosis fungoides (MF) and Sézary syndrome (SS) are the most common malignancies. Diagnosis requires the combination of clinical, pathologic, and molecular features. Significant advances have been made in understanding the genetic and epigenetic aberrations in SS and to some extent in MF. Several prognostic factors have been identified. The goal of treatment is to minimize morbidity and limit disease progression. However, hematopoietic stem cell transplantation, considered for patients with advanced stages, is the only therapy with curative intent.

Keywords: Cause; Cutaneous T-cell lymphoma; Diagnosis; Genetics; Mycosis fungoides; Review; Sézary syndrome; Therapy.

Publication types

  • Review

MeSH terms

  • Biopsy
  • Combined Modality Therapy / methods
  • Disease Management
  • Disease Susceptibility
  • Humans
  • Incidence
  • Mycosis Fungoides / diagnosis*
  • Mycosis Fungoides / epidemiology
  • Mycosis Fungoides / etiology
  • Mycosis Fungoides / therapy*
  • Neoplasm Staging
  • Phenotype
  • Prognosis
  • Sezary Syndrome / diagnosis*
  • Sezary Syndrome / epidemiology
  • Sezary Syndrome / etiology
  • Sezary Syndrome / therapy*
  • Skin / pathology
  • T-Lymphocytes / immunology
  • T-Lymphocytes / metabolism
  • T-Lymphocytes / pathology
  • Treatment Outcome