title: Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC).
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.
authors.affiliation: Duke University Medical Center, Durham, NC, USA. olsen001@mc.duke.edu
authors.forename: Elise A
authors.initials: EA
authors.lastname: Olsen
authors.forename: Alain H
authors.initials: AH
authors.lastname: Rook
authors.forename: John
authors.initials: J
authors.lastname: Zic
authors.forename: Youn
authors.initials: Y
authors.lastname: Kim
authors.forename: Pierluigi
authors.initials: P
authors.lastname: Porcu
authors.forename: Christiane
authors.initials: C
authors.lastname: Querfeld
authors.forename: Gary
authors.initials: G
authors.lastname: Wood
authors.forename: Marie-France
authors.initials: MF
authors.lastname: Demierre
authors.forename: Mark
authors.initials: M
authors.lastname: Pittelkow
authors.forename: Lynn D
authors.initials: LD
authors.lastname: Wilson
authors.forename: Lauren
authors.initials: L
authors.lastname: Pinter-Brown
authors.forename: Ranjana
authors.initials: R
authors.lastname: Advani
authors.forename: Sareeta
authors.initials: S
authors.lastname: Parker
authors.forename: Ellen J
authors.initials: EJ
authors.lastname: Kim
authors.forename: Jacqueline M
authors.initials: JM
authors.lastname: Junkins-Hopkins
authors.forename: Francine
authors.initials: F
authors.lastname: Foss
authors.forename: Patrick
authors.initials: P
authors.lastname: Cacchio
authors.forename: Madeleine
authors.initials: M
authors.lastname: Duvic
mesh_terms: D000477:Alkylating Agents / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D000970:Antineoplastic Agents / Q000627:therapeutic use; D002986:Clinical Trials as Topic; D003131:Combined Modality Therapy; D004359:Drug Therapy, Combination; D019317:Evidence-Based Medicine; D056572:Histone Deacetylase Inhibitors / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D008727:Methotrexate / Q000627:therapeutic use; D009182:Mycosis Fungoides / Q000473:pathology / Q000628:therapy; D011788:Quality of Life; D012176:Retinoids / Q000627:therapeutic use; D012751:Sezary Syndrome / Q000276:immunology / Q000473:pathology* / Q000628:therapy*; D012878:Skin Neoplasms / Q000276:immunology / Q000473:pathology* / Q000628:therapy*
chemical_list: D000477:Alkylating Agents; D000911:Antibodies, Monoclonal; D000970:Antineoplastic Agents; D056572:Histone Deacetylase Inhibitors; D007155:Immunologic Factors; D012176:Retinoids; D008727:Methotrexate