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
Authors: Scarisbrick Julia J. , Prince H. Miles , Vermeer Maarten H. , Quaglino Pietro , Horwitz Steven , Porcu Pierluigi , Stadler Rudolf , Wood Gary S. , Beylot-Barry Marie , Pham-Ledard Anne , Foss Francine , Girardi Michael , Bagot Martine , Michel Laurence , Battistella Maxime , Guitart Joan , Kuzel Timothy M. , Martinez-Escala Maria Estela , Estrach Teresa , Papadavid Evangelia , Antoniou Christina , Rigopoulos Dimitis , Nikolaou Vassilki , Sugaya Makoto , Miyagaki Tomomitsu , Gniadecki Robert , Sanches José Antonio , Cury-Martins Jade , Miyashiro Denis , Servitje Octavio , Muniesa Cristina , Berti Emilio , Onida Francesco , Corti Laura , Hodak Emilia , Amitay-Laish Iris , Ortiz-Romero Pablo L. , Rodríguez-Peralto Jose L. , Knobler Robert , Porkert Stefanie , Bauer Wolfgang , Pimpinelli Nicola , Grandi Vieri , Cowan Richard , Rook Alain , Kim Ellen , Pileri Alessandro , Patrizi Annalisa , Pujol Ramon M. , Wong Henry , Tyler Kelly , Stranzenbach Rene , Querfeld Christiane , Fava Paolo , Maule Milena , Willemze Rein , Evison Felicity , Morris Stephen , Twigger Robert , Talpur Rakhshandra , Kim Jinah , Ognibene Grant , Li Shufeng , Tavallaee Mahkam , Hoppe Richard T. , Duvic Madeleine , Whittaker Sean J. , Kim Youn H. 
Published: October 2015

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.