A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome

E. C. Benton, Siobhan Crichton, R. Talpur, N. S. Agar, P. A. Fields, E. Wedgeworth, T. J. Mitchell, M. Cox, S. Ferreira, P. Liu, A. Robson, E. Calonje, C. M. Stefanato, B. Wilkins, J. Scarisbrick, E. M. Wain, F. Child, S. Morris, M. Duvic, S. J. Whittaker*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

110 Citations (Scopus)

Abstract

Background: There is no prognostic index for primary cutaneous T-cell lymphomas such as mycosis fungoides (MF) and Sezary syndrome (SS).

Method: 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.

Findings: 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).

Interpretation: 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.

Original languageEnglish
Pages (from-to)2859-2868
Number of pages10
JournalEuropean Journal of Cancer
Volume49
Issue number13
DOIs
Publication statusPublished - Sept 2013

Keywords

  • Cutaneous lymphoma
  • Prognostic index
  • Mycosis fungoides
  • Sezary syndrome
  • T-CELL LYMPHOMA
  • LONG-TERM OUTCOMES
  • UNITED-STATES
  • EUROPEAN-ORGANIZATION
  • TUMOR BURDEN
  • TASK-FORCE
  • CLASSIFICATION
  • TRANSFORMATION
  • SURVIVAL
  • SOCIETY

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