Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC)

J Am Acad Dermatol. 2011 Feb;64(2):352-404. doi: 10.1016/j.jaad.2010.08.037. Epub 2010 Dec 9.


Sézary syndrome (SS) has a poor prognosis and few guidelines for optimizing therapy. The US Cutaneous Lymphoma Consortium, to improve clinical care of patients with SS and encourage controlled clinical trials of promising treatments, undertook a review of the published literature on therapeutic options for SS. An overview of the immunopathogenesis and standardized review of potential current treatment options for SS including metabolism, mechanism of action, overall efficacy in mycosis fungoides and SS, and common or concerning adverse effects is first discussed. The specific efficacy of each treatment for SS, both as monotherapy and combination therapy, is then reported using standardized criteria for both SS and response to therapy with the type of study defined by a modification of the US Preventive Services guidelines for evidence-based medicine. Finally, guidelines for the treatment of SS and suggestions for adjuvant treatment are noted.

Publication types

  • Review

MeSH terms

  • Alkylating Agents / therapeutic use
  • Antibodies, Monoclonal / therapeutic use
  • Antineoplastic Agents / therapeutic use
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Drug Therapy, Combination
  • Evidence-Based Medicine
  • Histone Deacetylase Inhibitors / therapeutic use
  • Humans
  • Immunologic Factors / therapeutic use
  • Methotrexate / therapeutic use
  • Mycosis Fungoides / pathology
  • Mycosis Fungoides / therapy
  • Quality of Life
  • Retinoids / therapeutic use
  • Sezary Syndrome / immunology
  • Sezary Syndrome / pathology*
  • Sezary Syndrome / therapy*
  • Skin Neoplasms / immunology
  • Skin Neoplasms / pathology*
  • Skin Neoplasms / therapy*


  • Alkylating Agents
  • Antibodies, Monoclonal
  • Antineoplastic Agents
  • Histone Deacetylase Inhibitors
  • Immunologic Factors
  • Retinoids
  • Methotrexate