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 Journal Article


Authors: Scarisbrick, J. J.; Prince, H. M.; Vermeer, M. H.; Quaglino, P.; Horwitz, S.; Porcu, P.; Stadler, R.; Wood, G. S.; Beylot-Barry, M.; Pham-Ledard, A.; Foss, F.; Girardi, M.; Bagot, M.; Michel, L.; Battistella, M.; Guitart, J.; Kuzel, T. M.; Martinez-Escala, M. E.; Estrach, T.; Papadavid, E.; Antoniou, C.; Rigopoulos, D.; Nikolaou, V.; Sugaya, M.; Miyagaki, T.; Gniadecki, R.; Sanches, J. A.; Cury-Martins, J.; Miyashiro, D.; Servitje, O.; Muniesa, C.; Berti, E.; Onida, F.; Corti, L.; Hodak, E.; Amitay-Laish, I.; Ortiz-Romero, P. L.; Rodríguez-Peralto, J. L.; Knobler, R.; Porkert, S.; Bauer, W.; Pimpinelli, N.; Grandi, V.; Cowan, R.; Rook, A.; Kim, E.; Pileri, A.; Patrizi, A.; Pujol, R. M.; Wong, H.; Tyler, K.; Stranzenbach, R.; Querfeld, C.; Fava, P.; Maule, M.; Willemze, R.; Evison, F.; Morris, S.; Twigger, R.; Talpur, R.; Kim, J.; Ognibene, G.; Li, S.; Tavallaee, M.; Hoppe, R. T.; Duvic, M.; Whittaker, S. J.; Kim, Y. H.
Article 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
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.
Keywords: adolescent; adult; cancer survival; child; aged; survival rate; major clinical study; overall survival; clinical feature; histopathology; advanced cancer; cancer risk; cancer staging; cell proliferation; skin; blood; cell transformation; lactate dehydrogenase; leukocyte count; cell clone; lactate dehydrogenase blood level; mycosis fungoides; lymphocyte count; cd30 antigen; sezary syndrome; health center; cancer prognosis; human; male; female; priority journal; article
Journal Title: Journal of Clinical Oncology
Volume: 33
Issue: 32
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 2015-11-10
Start Page: 3766
End Page: 3773
Language: English
DOI: 10.1200/JCO.2015.61.7142
PROVIDER: scopus
PUBMED: 26438120
PMCID: PMC4979132
DOI/URL:
Notes: Article -- Export Date: 7 January 2016 -- 3766 -- Source: Scopus
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MSK Authors
  1. Steven M Horwitz
    344 Horwitz