@article{3087595, title = "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", author = "Scarisbrick, J.J. and Prince, H.M. and Vermeer, M.H. and Quaglino, P. and Horwitz, S. and Porcu, P. and Stadler, R. and Wood, G.S. and Beylot-Barry, M. and Pham-Ledard, A. and Foss, F. and Girardi, M. and Bagot, M. and Michel, L. and Battistella, M. and Guitart, J. and Kuzel, T.M. and Martinez-Escala, M.E. and Estrach, T. and Papadavid, E. and Antoniou, C. and Rigopoulos, D. and Nikolaou, V. and Sugaya, M. and Miyagaki, T. and Gniadecki, R. and Sanches, J.A. and Cury-Martins, J. and Miyashiro, D. and Servitje, O. and Muniesa, C. and Berti, E. and Onida, F. and Corti, L. and Hodak, E. and Amitay-Laish, I. and Ortiz-Romero, P.L. and Rodríguez-Peralto, J.L. and Knobler, R. and Porkert, S. and Bauer, W. and Pimpinelli, N. and Grandi, V. and Cowan, R. and Rook, A. and Kim, E. and Pileri, A. and Patrizi, A. and Pujol, R.M. and Wong, H. and Tyler, K. and Stranzenbach, R. and Querfeld, C. and Fava, P. and Maule, M. and Willemze, R. and Evison, F. and Morris, S. and Twigger, R. and Talpur, R. and Kim, J. and Ognibene, G. and Li, S. and Tavallaee, M. and Hoppe, R.T. and Duvic, M. and Whittaker, S.J. and Kim, Y.H.", journal = "Journal of Clinical Oncology", year = "2015", volume = "33", number = "32", pages = "3766-3773", publisher = "American Society of Clinical Oncology", issn = "0732-183X, 1527-7755", doi = "10.1200/JCO.2015.61.7142", keywords = "CD30 antigen; lactate dehydrogenase; lactate dehydrogenase, adolescent; adult; advanced cancer; aged; Article; blood; cancer prognosis; cancer risk; cancer staging; cancer survival; cell clone; cell proliferation; cell transformation; child; clinical feature; female; health center; histopathology; human; lactate dehydrogenase blood level; leukocyte count; lymphocyte count; major clinical study; male; mycosis fungoides; overall survival; priority journal; Sezary syndrome; skin; survival rate; age; cancer staging; enzymology; Kaplan Meier method; metabolism; middle aged; mortality; mycosis fungoides; pathology; predictive value; prognosis; risk factor; Sezary syndrome; skin tumor; statistical model, Adult; Age Factors; Aged; Cell Transformation, Neoplastic; Female; Humans; Kaplan-Meier Estimate; L-Lactate Dehydrogenase; Male; Middle Aged; Models, Statistical; Mycosis Fungoides; Neoplasm Staging; Predictive Value of Tests; Prognosis; Risk Factors; Sezary Syndrome; Skin; Skin Neoplasms; Survival Rate", 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 IB, 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 surviva 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 ndex, may be useful to stratify advanced-stage patients. © 2015 by American Society of Clinical Oncology." }