A new prognostic index (CLIPI) for advanced cutaneous lymphoma enables precise patient risk stratification
Abstract
Abstract Advanced mycosis fungoides (MF) and Sézary syndrome (SS) have a poor overall survival (OS) of <5 years. Studies have found the current staging (IA-IVB) is inadequate for risk stratification. The PROCLIPI (Prospective Cutaneous Lymphoma International Prognostic Index) study was launched in 2015 at 46 international expert MF/SS centers, prospectively collecting predefined data sets in patients with newly diagnosed MF/SS, to determine a cutaneous lymphoma IPI (CLIPI). Five hundred fifty-two patients with advanced stage MF/SS were recruited. The 5-year OS was 50.0% for stage IIB, 64.8% for stage IIIA, 43.9% for stage IIIB, 50.8% for stage IVA1, 25.9% for stage IVA2, and 36.9% for stage IVB. Factors at diagnosis associated with a significantly worse survival were N3 status (P < .001), age >60 years (P < .001), raised serum lactate dehydrogenase (P = .005), and large-cell transformation in skin (P = .006). Modeling these 4 independent risk factors into a CLIPI found that there was a worse OS in high- vs low-risk (P < .001), high- vs intermediate-risk (P = .002) and intermediate- vs low-risk (P = .010) groups. Five-year OS was 63.3%, 44.7%, and 18.3% in the low-, intermediate-, and high-risk groups, respectively. In this advanced stage cohort there was a low 5-year survival and increasing stage was not associated with worsening survival. The use of CLIPI to stratify patients into risk groups has the potential to improve outcomes and aid optimal treatment selection. This trial was registered at www.ClinicalTrials.gov as #NCT02848274.
Article Details
Authors (55)
Julia J. Scarisbrick
1Department of Dermatology, University Hospitals Birmingham National Health Service (NHS) Foundation Trust and University of Birmingham, Birmingham, United Kingdom
Pietro Quaglino
14University of Turin, Department of Medical Sciences, Dermatologic Clinic, Turin, Italy
Sean Whittaker
3St Johns Institute of Dermatology, King’s College London, London, United Kingdom
Martine Bagot
4Hôpital Saint Louis, APHP, Inserm U976, Université Paris Cité, Paris, France
Emmanuella Guenova
5Department of Dermatology, University of Lausanne and Lausanne University Hospital, Lausanne, Switzerland
Evangelia Papadavid
7Epworth Healthcare, Peter McCallum Cancer Center, The University of Melbourne, Monash University, Melbourne, Australia
Henry Miles Prince
8Department of Dermatology, University of Sao Paulo Medical School, Sao Paulo, Brazil
Jose A. Sanches
9Department of Dermatology, Hospital Universitari de Bellvitge, Barcelona, Spain
Denis R. Miyashiro
9Department of Dermatology, Hospital Universitari de Bellvitge, Barcelona, Spain
Octavio Servitje
10City of Hope Comprehensive Cancer Center and Beckman Research Institute, Duarte, CA
Christiane Querfeld
4City of Hope, Duarte, United States
Oleg Akilov
9University of Pittsburgh Medical Center, Pittsburgh, United States
Arvind Arumainathan
13University Hospital Southampton, Southampton, United Kingdom
Luke Bennett
Maxime Battistella
5Assistance Publique des Hôpitaux de Paris (APHP), Hôpital Saint Louis, Service d'anatomopathologie, Paris, France
Kim Benstead
15Department of Dermatology, University of Milan, Milan, Italy
Emilio Berti
Marie Beylot-Barry
Department of Dermatology, INSERM Unité 1312, Centre Hospitalier Universitaire (CHU) and Université de Bordeaux, Bordeaux, France
Anne-Marie Busschots
18The Christie Hospital, Manchester, United Kingdom
Richard Cowan
6Christie Hospital Foundation NHS Trust, University of Manchester, Manchester, United Kingdom
Reinhard Dummer
Giles Dunnill
21Hospital Clinic, University of Barcelona, Barcelona, Spain
Teresa Estrach
22Columbia University Medical Center, New York, NY
Felicity Evison
1Department of Dermatology, University Hospitals Birmingham National Health Service (NHS) Foundation Trust and University of Birmingham, Birmingham, United Kingdom
Abraham Bashir
1University Hospitals Birmingham NHS Foundation Trust, Department of Dermatology, Birmingham, United Kingdom
Larisa Geskin
3Columbia University Vagelos College of Physicians and Surgeons, New York, United States
Ale Gru
23Departments of Dermatology and Pathology, Northwestern University, Chicago, IL
Joan Guitart
2Northwestern University Feinberg School of Medicine, Dermatology, Chicago, United States
Melba C. Herrera
25Cutaneous Lymphoma Unit, Davidoff Cancer Center, Beilinson Hospital, Tel Aviv University, Tel Aviv Rabin Medical Center, Tel Aviv, Israel
Emillia Hodak
26Memorial Sloan Kettering Cancer Center, New York, NY
Steve Horwitz
27University of Vienna Medical School, Vienna, Austria
Constanze Jonak
Claus-Detlev Klemke
29Beatson West of Scotland Cancer Center, Glasgow, United Kingdom
Robert Knobler
28Department of Dermatology, Akademisches Lehrkrankenhaus der Universität Freiburg, Karlsruhe, Germany
Pam Mckay
30Department of Dermato-venereology, Semmelweis Medical University, Budapest, Hungary
Marta Marschalkó
31Dermatology Unit, Galliera Hospital, Genoa, Italy
Cesare Massone
32Oxford University Hospitals NHS Trust, Oxford, United Kingdom
Rubeta Matin
33Dermatopathology and Cutaneous Lymphoma Unit, Department of Dermatology, Venereology und Allergology, HELIOS Klinikum Hildesheim GmbH, Hildesheim, Germany
Christina Mitteldorf
34Department of Pathology, Stanford University School of Medicine, Palo Alto, CA
Robert Novoa
25Cutaneous Lymphoma Unit, Davidoff Cancer Center, Beilinson Hospital, Tel Aviv University, Tel Aviv Rabin Medical Center, Tel Aviv, Israel
Pablo Ortiz-Romero
11Institute i+12, Hospital 12 de Octubre Medical School, University Complutense, Department of Dermatology, Madrid, Spain
Nicola Pimpinelli
37Dermatology Department, Hospital del Mar, Barcelona, Spain
Ramon M. Pujol
38Department of Skin and Allergic Diseases, University of Helsinki and Helsinki University Central Hospital, Helsinki, Finland
Annmari Ranki
39Cutaneous Lymphoma Centre, Department of Dermatology, Johannes Wesling Medical Centre, University Hospitals of the Ruhr University of Bochum, Minden, Germany
Liisa Vakeva
39Cutaneous Lymphoma Centre, Department of Dermatology, Johannes Wesling Medical Centre, University Hospitals of the Ruhr University of Bochum, Minden, Germany
Kerri E. Rieger
Department of Dermatology, Stanford University School of Medicine
Rudolf Stadler
40Department of Dermatology and Venereology, University Hospital of St. Pölten, Karl Landsteiner University of Health Sciences, St Pölten, Austria
Franz Trautinger
41Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands
Johanna Latzka
41Department of Dermatology, Leiden University Medical Center, Leiden, The Netherlands
Maarten Vermeer
Rachel Wachsmuth
43Newcastle Upon Tyne NHS Trust, Newcastle, United Kingdom
Sophie Weatherhead
44Department of Dermatology Medical Department II, Christian-Albrechts-University Kiel, University Hospital of Munich, Munich, Germany
Ulrike Wehkamp
45Department of Dermatology, Venereology and Allergology, University Hospital Wuerzburg, Wuerzburg, Germany
Marion Wobser
46University Institute and Clinic for Immunodermatology, Medical Faculty, Johannes Kepler University, Linz, Austria
Youn H. Kim
25Cutaneous Lymphoma Unit, Davidoff Cancer Center, Beilinson Hospital, Tel Aviv University, Tel Aviv Rabin Medical Center, Tel Aviv, Israel