Cutaneous Lymphoma International Prognostic Index and Risk Stratification in Advanced Mycosis Fungoides and Sézary Syndrome

Importance 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. Objective 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. Design, Setting, and Participants 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. Exposure 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). Main Outcomes and Measures 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. Results 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). Conclusions and Relevance 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.

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Journal
JAMA Dermatology
Published
2026-10-07
DOI
https://doi.org/10.1001/jamadermatol.2026.3856
Citations
1
Primary Topic
Cutaneous lymphoproliferative disorders research
Type
article
Field-Weighted Citation Impact
6.58
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article

Cutaneous Lymphoma International Prognostic Index and Risk Stratification in Advanced Mycosis Fungoides and Sézary Syndrome

Nicola Pimpinelli, Kerri E. Rieger, Christoph Iselin, José Antônio Sanches et al.
1 citations
JAMA Dermatology
Cutaneous lymphoproliferative disorders research
6.58
article

Cutaneous Lymphoma International Prognostic Index and Risk Stratification in Advanced Mycosis Fungoides and Sézary Syndrome

Nicola Pimpinelli, Kerri E. Rieger, Christoph Iselin, José Antônio Sanches, Alejandro Ariel Gru, Gabriele Roccuzzo, Luke Bennett, Márta Marschalkó, Marion Wobser, Rubeta Matin, Johanna Latzka, Ulrike Wehkamp, Emmilia Hodak, Mike Bayne, Christina Mitteldorf, Pietro Quaglino, Evangelia Papadavid, Emmanuella Guenova, Franz Trautinger, Denis Miyashiro, Maxime Battistella, Octavio Servitje, Felicity Evison, Youn H. Kim, Melba Herrera, Anne Marie Busschots, Claus‐Detlev Klemke, Marie Beylot-Barry, Pablo Ortiz‐Romero, Sophie Weatherhead, Pam McKay, Abraham Bashir, Giles Dunnill, Richard Cowan, Rachel Wachsmuth, Henry Miles Prince, Julia Scarisbrick, Larisa Geskin, Oleg Akilov, Martine Bagot, Silvia Alberti Violetti, Robert Novoa, Rudolf Stadler, Christiane Querfeld, Cesare Massone, Reinhard Dummer, Arvind Arumainathan, Steve Horwitz, Paolo Fava, Sean Whittaker, Robert Knobler, Ramon M. Pujol, Teresa Estrach, Constanze Jonak, Joan Guitart, Maarten Vermeer
article en
1 citations

Abstract

Importance 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. Objective 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. Design, Setting, and Participants 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. Exposure 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). Main Outcomes and Measures 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. Results 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). Conclusions and Relevance 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.

JAMA Dermatology
Semmelweis University (HU), Northwestern University (US), Johannes Kepler University of Linz (AT), City Of Hope National Medical Center (US), University Hospitals Birmingham NHS Foundation Trust (GB), Memorial Sloan Kettering Cancer Center (US), Université de Bordeaux (FR), Inserm (FR), King's College - North Carolina (US), University of Pittsburgh (US), Universidade de São Paulo (BR), National and Kapodistrian University of Athens (GR), University of Zurich (CH), Université Paris Cité (FR), Royal Devon & Exeter NHS Foundation Trust (GB), Columbia University Irving Medical Center (US), University Hospitals of the Ruhr-University of Bochum (DE), Epworth Hospital (AU), Leiden University Medical Center (NL), Newcastle City Council (GB), Universitair Ziekenhuis Leuven (BE), Beatson West of Scotland Cancer Centre (GB), Rabin Medical Center (IL), Peter MacCallum Cancer Centre (AU), Newcastle upon Tyne Hospitals NHS Foundation Trust (GB), Bellvitge University Hospital (ES), Gloucestershire Hospitals NHS Foundation Trust (GB), Bristol Royal Infirmary (GB), University Hospital Southampton NHS Foundation Trust (GB), Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico (IT), Clatterbridge Cancer Centre NHS Foundation Trust (GB), Ente Ospedaliero Ospedali Galliera (IT), Research Institute Hospital 12 de Octubre (ES), MSH Medical School Hamburg – University of Applied Sciences and Medical University (DE), Assistance Publique – Hôpitaux de Paris (FR), Northwestern University (PH), University Hospital of Zurich (CH), Bordeaux Population Health (FR), City of Hope (US), Universitätsklinikum St. Pölten (AT), Universitätsmedizin Göttingen (DE), Kepler Universitätsklinikum (AT), Taunton & Somerset NHS Foundation Trust (GB), Newcastle upon Tyne Hospital (GB), Universitätsklinikum Würzburg (DE), UPMC Hillman Cancer Center (US), Hospital Del Mar (ES), The Christie Hospital (GB), University of Pittsburgh Medical Center (US), Stanford Medicine (US), Helios Kliniken (DE), Hôpital Saint-Louis (FR), Northwestern Medicine (US), Oxford University Hospitals NHS Trust (GB), St. Josefskrankenhaus Freiburg (DE), Kantonsspital Aarau (CH), University of Southampton (GB), Beckman Research Institute, University of Florence (IT), University of Turin (IT), Universitat de Barcelona (ES), University of Göttingen (DE), Medical University of Vienna (AT), Stanford University (US), University of Lausanne (CH)
Openalex Percentile: Top 3%
Cutaneous lymphoproliferative disorders research
6.58
DOISourceOpenAlex
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