title: Survival outcomes and prognostic factors in mycosis fungoides/Sézary syndrome: validation of the revised International Society for Cutaneous Lymphomas/European Organisation for Research and Treatment of Cancer staging proposal.
abstract: OBJECTIVE
  We have analyzed the outcome of mycosis fungoides (MF) and Sézary syndrome (SS) patients using the recent International Society for Cutaneous Lymphomas (ISCL)/European Organisation for Research and Treatment of Cancer (EORTC) revised staging proposal.
  
  
  METHODS
  Overall survival (OS), disease-specific survival (DSS), and risk of disease progression (RDP) were calculated for a cohort of 1,502 patients using univariate and multivariate models.
  
  
  RESULTS
  The mean age at diagnosis was 54 years, and 71% of patients presented with early-stage disease. Disease progression occurred in 34%, and 26% of patients died due to MF/SS. A significant difference in survival and progression was noted for patients with early-stage disease having patches alone (T1a/T2a) compared with those having patches and plaques (T1b/T2b). Univariate analysis established that (1) advanced skin and overall clinical stage, increased age, male sex, increased lactate dehydrogenase (LDH), and large-cell transformation were associated with reduced survival and increased RDP; (2) hypopigmented MF, MF with lymphomatoid papulosis, and poikilodermatous MF were associated with improved survival and reduced RDP; and (3) folliculotropic MF was associated with an increased RDP. Multivariate analysis established that (1) advanced skin (T) stage, the presence in peripheral blood of the tumor clone without Sézary cells (B0b), increased LDH, and folliculotropic MF were independent predictors of poor survival and increased RDP; (2) large-cell transformation and tumor distribution were independent predictors of increased RDP only; and (3) N, M, and B stages; age; male sex; and poikilodermatous MF were only significant for survival.
  
  
  CONCLUSIONS
  This study has validated the recently proposed ISCL/EORTC staging system and identified new prognostic factors.
authors.affiliation: Guy's and St Thomas' National Health Service (NHS) Foundation Trust, London, United Kingdom.
authors.forename: Nita Sally
authors.initials: NS
authors.lastname: Agar
authors.forename: Emma
authors.initials: E
authors.lastname: Wedgeworth
authors.forename: Siobhan
authors.initials: S
authors.lastname: Crichton
authors.forename: Tracey J
authors.initials: TJ
authors.lastname: Mitchell
authors.forename: Michael
authors.initials: M
authors.lastname: Cox
authors.forename: Silvia
authors.initials: S
authors.lastname: Ferreira
authors.forename: Alistair
authors.initials: A
authors.lastname: Robson
authors.forename: Eduardo
authors.initials: E
authors.lastname: Calonje
authors.forename: Catherine M
authors.initials: CM
authors.lastname: Stefanato
authors.forename: Elizabeth Mary
authors.initials: EM
authors.lastname: Wain
authors.forename: Bridget
authors.initials: B
authors.lastname: Wilkins
authors.forename: Paul A
authors.initials: PA
authors.lastname: Fields
authors.forename: Alan
authors.initials: A
authors.lastname: Dean
authors.forename: Katherine
authors.initials: K
authors.lastname: Webb
authors.forename: Julia
authors.initials: J
authors.lastname: Scarisbrick
authors.forename: Stephen
authors.initials: S
authors.lastname: Morris
authors.forename: Sean J
authors.initials: SJ
authors.lastname: Whittaker
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000704:Analysis of Variance; D014408:Biomarkers, Tumor / Q000097:blood; D001706:Biopsy; D002471:Cell Transformation, Neoplastic / Q000473:pathology; D018450:Disease Progression; D018572:Disease-Free Survival; D005060:Europe; D005260:Female; D018859:Hair Follicle / Q000473:pathology; D006801:Humans; D007391:International Cooperation; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008198:Lymph Nodes / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009182:Mycosis Fungoides / Q000401:mortality / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging / Q000379:methods* / Q000592:standards; D011379:Prognosis; D012044:Regression Analysis; D018570:Risk Assessment; D012307:Risk Factors; D012737:Sex Factors; D012751:Sezary Syndrome / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012878:Skin Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012955:Societies, Medical; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D014408:Biomarkers, Tumor; D007770:L-Lactate Dehydrogenase