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.
Keywords
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Article info
Publication history
Published online: June 03, 2013
Identification
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© 2013 Published by Elsevier Inc. All rights reserved.
