Authors
Gabriele Roccuzzo, Abraham Bashir, Felicity Evison, Sean Whittaker, Martine Bagot, Emmanuella Guenova, Evangelia Papadavid, Henry Miles Prince, Jose A Sanches, Denis R Miyashiro, Octavio Servitje, Christiane Querfeld, Oleg Akilov, Arvind Arumainathan, Luke Bennett, Maxime Battistella, Mike Bayne, Silvia Alberti Violetti, Paolo Fava, Christoph Iselin, Marie Beylot-Barry, Anne Marie Busschots, Richard Cowan, Reinhard Dummer, Giles Dunnill, Teresa Estrach, Larisa Geskin, Alejandro A Gru, Joan Guitart, Melba C Herrera, Emmilia Hodak, Steve Horwitz, Constanze Jonak, Claus-Detlev Klemke, Robert Knobler, Pam Mckay, Marta Marschalkó, Cesare Massone, Rubeta N Matin, Christina Mitteldorf, Robert Novoa, Pablo Ortiz-Romero, Nicola Pimpinelli, Ramon M Pujol, Kerri E Rieger, Rudolf Stadler, Franz Trautinger, Johanna Latzka, Maarten Vermeer, Rachel Wachsmuth, Sophie Weatherhead, Ulrike Wehkamp, Marion Wobser, Pietro Quaglino, Youn H Kim, Julia Scarisbrick
Published in
JAMA dermatology. Oct 07, 2026. Epub Oct 07, 2026.
Abstract
Risk stratification in advanced mycosis fungoides and Sézary syndrome (MF/SS) remains suboptimal given that current TNMB (tumor, node, metastasis, blood) staging has limited prognostic value for clinically relevant outcomes.
To determine whether the Cutaneous Lymphoma International Prognostic Index (CLIPI) may be used to predict progression-free survival (PFS) and best objective response (BOR) in patients with advanced MF/SS.
This international prospective cohort study included patients with stage IIB to IVB MF/SS from the PROCLIPI trial which ran from July 1, 2015, to May 1, 2025, across 46 referral centers. Median follow-up was 4.75 (95% CI, 4.04-5.13) years; eligible participants had complete data on CLIPI, PFS, and BOR. Data were analyzed from September 1 to December 1, 2025.
CLIPI score at diagnosis, based on age (>60 years), elevated serum lactate dehydrogenase, N3 nodal status, and large-cell transformation in the skin. Patients were classified as having low (0 or 1 factor), intermediate (2 factors), or high risk (3 or 4 factors).
PFS (defined as diagnosis to disease progression or death) and BOR (complete response, partial response, stable disease, or progressive disease) during follow-up. Exploratory analyses evaluated treatment effects before progression using inverse probability weighting.
Among 277 patients (median [IQR] age at diagnosis, 66 [23-89] years; 183 male individuals [65.7%]), 144 (52.0%) were classified as having low risk, 93 (33.6%) intermediate risk, and 40 (14.4%) high risk. They had a median PFS of 4.64 years (95% CI, 3.50-5.76), 2.72 years (95% CI, 1.81-4.56), and 0.89 years (95% CI, 0.65-1.14), respectively (log-rank P < .001). Patients in the intermediate-risk (hazard ratio [HR], 1.45; 95% CI, 1.01-2.08) and high-risk (HR, 3.84; 95% CI, 2.21-6.66) CLIPI groups displayed higher hazards of progression or death than those at low risk. Inclusion of CLIPI improved PFS discrimination over a model with baseline stage alone. Each 1-point increase in CLIPI was associated with approximately 38% lower odds of observing a better response during follow-up (odds ratio, 0.62; 95% CI, 0.43-0.89).
In this international prospective cohort of patients with advanced MF/SS, CLIPI scores were associated with risk of disease progression and likelihood of response beyond clinical stage. These findings indicate that CLIPI may help to refine risk stratification and support its integration into clinical decision-making and future trial design.
PMID:
42842270
Bibliographic data and abstract were imported from PubMed on 08 Oct 2026.
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