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. 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

Julia J Scarisbrick  1 , H Miles Prince  1 , Maarten H Vermeer  1 , Pietro Quaglino  1 , Steven Horwitz  1 , Pierluigi Porcu  1 , Rudolf Stadler  1 , Gary S Wood  1 , Marie Beylot-Barry  1 , Anne Pham-Ledard  1 , Francine Foss  1 , Michael Girardi  1 , Martine Bagot  1 , Laurence Michel  1 , Maxime Battistella  1 , Joan Guitart  1 , Timothy M Kuzel  1 , Maria Estela Martinez-Escala  1 , Teresa Estrach  1 , Evangelia Papadavid  1 , Christina Antoniou  1 , Dimitis Rigopoulos  1 , Vassilki Nikolaou  1 , Makoto Sugaya  1 , Tomomitsu Miyagaki  1 , Robert Gniadecki  1 , José Antonio Sanches  1 , Jade Cury-Martins  1 , Denis Miyashiro  1 , Octavio Servitje  1 , Cristina Muniesa  1 , Emilio Berti  1 , Francesco Onida  1 , Laura Corti  1 , Emilia Hodak  1 , Iris Amitay-Laish  1 , Pablo L Ortiz-Romero  1 , Jose L Rodríguez-Peralto  1 , Robert Knobler  1 , Stefanie Porkert  1 , Wolfgang Bauer  1 , Nicola Pimpinelli  1 , Vieri Grandi  1 , Richard Cowan  1 , Alain Rook  1 , Ellen Kim  1 , Alessandro Pileri  1 , Annalisa Patrizi  1 , Ramon M Pujol  1 , Henry Wong  1 , Kelly Tyler  1 , Rene Stranzenbach  1 , Christiane Querfeld  1 , Paolo Fava  1 , Milena Maule  1 , Rein Willemze  1 , Felicity Evison  1 , Stephen Morris  1 , Robert Twigger  1 , Rakhshandra Talpur  1 , Jinah Kim  1 , Grant Ognibene  1 , Shufeng Li  1 , Mahkam Tavallaee  1 , Richard T Hoppe  1 , Madeleine Duvic  1 , Sean J Whittaker  1 , Youn H Kim  1
Affiliations

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

Julia J Scarisbrick et al. J Clin Oncol. .

Abstract

Purpose: 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.

Patients and methods: 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).

Results: 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%).

Conclusion: 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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Conflict of interest statement

Authors' disclosures of potential conflicts of interest are found in the article online at www.jco.org. Author contributions are found at the end of this article.

Figures

Fig 1.
Fig 1.
Kaplan-Meier plot showing survival by stage.
Fig 2.
Fig 2.
Kaplan-Meier plot showing prognostic index model for low-, intermediate-, and high-risk groups. Variables included in the prognostic index model were stage IV, elevated lactate dehydrogenase, age greater than 60 years, and large-cell transformation in skin (low risk = zero to one variable; intermediate risk = two variables; high risk = three to four variables).
Fig A1.
Fig A1.
Kaplan-Meier plot showing survival according to blood (B) classification.
Fig A2.
Fig A2.
Kaplan-Meier plot showing survival by revised staging.
See this image and copyright information in PMC

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