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J Clin Oncol. 2015 Nov 10;33(32):3766-73. doi: 10.1200/JCO.2015.61.7142. Epub 2015 Oct 5.

Cutaneous Lymphoma International Consortium Study of Outcome in Advanced Stages of Mycosis Fungoides and Sézary Syndrome: Effect of Specific Prognostic Markers on Survival and Development of a Prognostic Model.

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Julia J. Scarisbrick and Felicity Evison, University Hospital Birmingham, Birmingham; Richard Cowan, The Christie Hospital, Manchester; Stephen Morris and Sean J. Whittaker, St Thomas' Hospital, London, United Kingdom; H. Miles Prince and Robert Twigger, Peter MacCallum Cancer Centre, East Melbourne, Victoria, Australia; Maarten H. Vermeer and Rein Willemze, Leiden University Medical Centre, Leiden, the Netherlands; Pietro Quaglino, Paolo Fava, and Milena Maule, University of Turin (Torino), Turin; Emilio Berti, Francesco Onida, and Laura Corti, Fondazione Istituto di Ricovero e Cura a Carattere Scientifico Ca' Granda Ospedale Maggiore Policlinico, University of Milano-Bicocca, Milan; Nicola Pimpinelli and Vieri Grandi, University of Florence, Florence; Alessandro Pileri and Annalisa Patrizi, University of Bologna, Bologna, Italy; Steven Horwitz and Christiane Querfeld, Memorial Sloan-Kettering Cancer Center, New York, NY; Pierluigi Porcu, Henry Wong, and Kelly Tyler, Ohio State University, Columbus, OH; Gary S. Wood, University of Wisconsin, Madison, WI; Francine Foss and Michael Girardi, Yale University, New Haven, CT; Joan Guitart, Timothy M. Kuzel, and Maria Estela Martinez-Escala, Northwestern University, Chicago, IL; Alain Rook and Ellen Kim, University of Pennsylvania, Philadelphia; PA; Christiane Querfeld, City of Hope Hospital, Duarte; Jinah Kim, Grant Ognibene, Shufeng Li, Mahkam Tavallaee, Richard T. Hoppe, and Youn H. Kim, Stanford University Medical Center, Stanford, CA; Rakhshandra Talpur and Madeleine Duvic, The University of Texas MD Anderson Cancer Center, Houston, TX; Rudolf Stadler and Rene Stranzenbach, Unit University Munster, Minden, Germany; Marie Beylot-Barry and Anne Pham-Ledard, Centre Hospitalier Universitaire Hospital de Bordeaux, Bordeaux; Martine Bagot, Laurence Michel, and Maxime Battistella, Hospital St Louis, Paris, France; Teresa Estrach, Hospital Clínico, University of Barcelona, Villarroel; Octavio Servitje and Cristina Muniesa,



Advanced-stage mycosis fungoides (MF; stage IIB to IV) and Sézary syndrome (SS) are aggressive lymphomas with a median survival of 1 to 5 years. Clinical management is stage based; however, there is wide range of outcome within stages. Published prognostic studies in MF/SS have been single-center trials. Because of the rarity of MF/SS, only a large collaboration would power a study to identify independent prognostic markers.


Literature review identified the following 10 candidate markers: stage, age, sex, cutaneous histologic features of folliculotropism, CD30 positivity, proliferation index, large-cell transformation, WBC/lymphocyte count, serum lactate dehydrogenase, and identical T-cell clone in blood and skin. Data were collected at specialist centers on patients diagnosed with advanced-stage MF/SS from 2007. Each parameter recorded at diagnosis was tested against overall survival (OS).


Staging data on 1,275 patients with advanced MF/SS from 29 international sites were included for survival analysis. The median OS was 63 months, with 2- and 5-year survival rates of 77% and 52%, respectively. The median OS for patients with stage IIB disease was 68 months, but patients diagnosed with stage III disease had slightly improved survival compared with patients with stage IIB, although patients diagnosed with stage IV disease had significantly worse survival (48 months for stage IVA and 33 months for stage IVB). Of the 10 variables tested, four (stage IV, age > 60 years, large-cell transformation, and increased lactate dehydrogenase) were independent prognostic markers for a worse survival. Combining these four factors in a prognostic index model identified the following three risk groups across stages with significantly different 5-year survival rates: low risk (68%), intermediate risk (44%), and high risk (28%).


To our knowledge, this study includes the largest cohort of patients with advanced-stage MF/SS and identifies markers with independent prognostic value, which, used together in a prognostic index, may be useful to stratify advanced-stage patients.

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