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

Elise A. Olsen, Alain H. Rook, John Zic, Youn Kim, Pierluigi Porcu, Christiane Querfeld, Gary Wood, Marie France Demierre, Mark Pittelkow, Lynn D. Wilson, Lauren Pinter-Brown, Ranjana Advani, Sareeta Parker, Ellen J. Kim, Jacqueline M. Junkins-Hopkins, Francine Foss, Patrick Cacchio, Madeleine Duvic

Research output: Contribution to journalReview articlepeer-review

146 Scopus citations

Abstract

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.

Original languageEnglish (US)
Pages (from-to)352-404
Number of pages53
JournalJournal of the American Academy of Dermatology
Volume64
Issue number2
DOIs
StatePublished - Feb 2011

Keywords

  • Sézary syndrome

ASJC Scopus subject areas

  • Dermatology

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