title: A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome.
abstract: BACKGROUND
  There is no prognostic index for primary cutaneous T-cell lymphomas such as mycosis fungoides (MF) and Sezary syndrome (SS).
  
  
  METHODS
  Two prognostic indices were developed for early (IA-IIA) and late stage (IIB-IVB) disease based on multivariate data from 1502 patients. End-points included overall survival (OS) and progression free survival (PFS). External validation included 1221 patients.
  
  
  RESULTS
  Significant adverse prognostic factors at diagnosis consisted of male gender, age >60, plaques, folliculotropic disease and stage N1/Nx for early stage, and male gender, age >60, stages B1/B2, N2/3 and visceral involvement for late stage disease. Using these variables we constructed two separate models each defined using 3 distinct groups for early and late stage patients: 0-1 (low risk), 2 (intermediate risk), and 3-5 factors (high risk). 10 year OS in the early stage model was 90.3% (low), 76.2% (intermediate) and 48.9% (high) and for the late stage model 53.2% (low), 19.8% (intermediate) and 15.0% (high). For the validation set significant differences in OS and PFS in early stage patients (both p<0.001) were also noted. In late stage patients, only OS differed between the groups (p=0.002).
  
  
  CONCLUSIONS
  This proposed cutaneous lymphoma prognostic index provides a model for prediction of OS in early and late stage MF/SS enabling rational therapeutic choices and patient stratification in clinical trials.
authors.affiliation: St. John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.
authors.forename: E C
authors.initials: EC
authors.lastname: Benton
authors.forename: S
authors.initials: S
authors.lastname: Crichton
authors.forename: R
authors.initials: R
authors.lastname: Talpur
authors.forename: N S
authors.initials: NS
authors.lastname: Agar
authors.forename: P A
authors.initials: PA
authors.lastname: Fields
authors.forename: E
authors.initials: E
authors.lastname: Wedgeworth
authors.forename: T J
authors.initials: TJ
authors.lastname: Mitchell
authors.forename: M
authors.initials: M
authors.lastname: Cox
authors.forename: S
authors.initials: S
authors.lastname: Ferreira
authors.forename: P
authors.initials: P
authors.lastname: Liu
authors.forename: A
authors.initials: A
authors.lastname: Robson
authors.forename: E
authors.initials: E
authors.lastname: Calonje
authors.forename: C M
authors.initials: CM
authors.lastname: Stefanato
authors.forename: B
authors.initials: B
authors.lastname: Wilkins
authors.forename: J
authors.initials: J
authors.lastname: Scarisbrick
authors.forename: E M
authors.initials: EM
authors.lastname: Wain
authors.forename: F
authors.initials: F
authors.lastname: Child
authors.forename: S
authors.initials: S
authors.lastname: Morris
authors.forename: M
authors.initials: M
authors.lastname: Duvic
authors.forename: S J
authors.initials: SJ
authors.lastname: Whittaker
mesh_terms: D014408:Biomarkers, Tumor / Q000097:blood; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; D007770:L-Lactate Dehydrogenase / Q000097:blood; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D009182:Mycosis Fungoides / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D009367:Neoplasm Staging; D016016:Proportional Hazards Models; D012307:Risk Factors; D012751:Sezary Syndrome / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D012878:Skin Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy; D013997:Time Factors
chemical_list: D014408:Biomarkers, Tumor; D007770:L-Lactate Dehydrogenase
keywords: Cutaneous lymphoma; Mycosis fungoides; Prognostic index; Sezary syndrome