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A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome.
Eur J Cancer 2013; 49(13):2859-68EJ

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.

Authors+Show Affiliations

St. John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust, London, UK.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

23735705

Citation

Benton, E C., et al. "A Cutaneous Lymphoma International Prognostic Index (CLIPi) for Mycosis Fungoides and Sezary Syndrome." European Journal of Cancer (Oxford, England : 1990), vol. 49, no. 13, 2013, pp. 2859-68.
Benton EC, Crichton S, Talpur R, et al. A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. Eur J Cancer. 2013;49(13):2859-68.
Benton, E. C., Crichton, S., Talpur, R., Agar, N. S., Fields, P. A., Wedgeworth, E., ... Whittaker, S. J. (2013). A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. European Journal of Cancer (Oxford, England : 1990), 49(13), pp. 2859-68. doi:10.1016/j.ejca.2013.04.018.
Benton EC, et al. A Cutaneous Lymphoma International Prognostic Index (CLIPi) for Mycosis Fungoides and Sezary Syndrome. Eur J Cancer. 2013;49(13):2859-68. PubMed PMID: 23735705.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - A cutaneous lymphoma international prognostic index (CLIPi) for mycosis fungoides and Sezary syndrome. AU - Benton,E C, AU - Crichton,S, AU - Talpur,R, AU - Agar,N S, AU - Fields,P A, AU - Wedgeworth,E, AU - Mitchell,T J, AU - Cox,M, AU - Ferreira,S, AU - Liu,P, AU - Robson,A, AU - Calonje,E, AU - Stefanato,C M, AU - Wilkins,B, AU - Scarisbrick,J, AU - Wain,E M, AU - Child,F, AU - Morris,S, AU - Duvic,M, AU - Whittaker,S J, Y1 - 2013/06/01/ PY - 2013/04/05/received PY - 2013/04/22/revised PY - 2013/04/23/accepted PY - 2013/6/6/entrez PY - 2013/6/6/pubmed PY - 2013/10/18/medline KW - Cutaneous lymphoma KW - Mycosis fungoides KW - Prognostic index KW - Sezary syndrome SP - 2859 EP - 68 JF - European journal of cancer (Oxford, England : 1990) JO - Eur. J. Cancer VL - 49 IS - 13 N2 - 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. SN - 1879-0852 UR - https://www.unboundmedicine.com/medline/citation/23735705/A_cutaneous_lymphoma_international_prognostic_index__CLIPi__for_mycosis_fungoides_and_Sezary_syndrome_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0959-8049(13)00352-3 DB - PRIME DP - Unbound Medicine ER -