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

Journal Article (Journal Article;Review)

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.

Full Text

Duke Authors

Cited Authors

  • Olsen, EA; Rook, AH; Zic, J; Kim, Y; Porcu, P; Querfeld, C; Wood, G; Demierre, M-F; Pittelkow, M; Wilson, LD; Pinter-Brown, L; Advani, R; Parker, S; Kim, EJ; Junkins-Hopkins, JM; Foss, F; Cacchio, P; Duvic, M

Published Date

  • February 2011

Published In

Volume / Issue

  • 64 / 2

Start / End Page

  • 352 - 404

PubMed ID

  • 21145619

Electronic International Standard Serial Number (EISSN)

  • 1097-6787

Digital Object Identifier (DOI)

  • 10.1016/j.jaad.2010.08.037


  • eng

Conference Location

  • United States