Julia Scarisbrick, Pietro Quaglino, Miles Prince, Maarten Vermeer, Evangelina Papadavid, Emilia Hodak, Sean Whittaker, Martine Bagot, Christina Querfeld, Oleg Akilov, Octavio Servitje, Emilio Berti, Pablo Ortiz-Romero, Rudolf Stadler, Constanze Jonak, Robert Knobler, Christina Mitteldorf, Teresa Estrach, Marta Marschalko, Emmanuella Guenova, Nicola Pimpinelli, Marie Beylot-Barry, Marion Wobser, Ulrike Wehkamp, Richard Cowan, Liisa Vakeva, Anne-Marie Buschots, Rubeta Matin, Felicity Evison, Lorenzo Cerroni, Werner Kempf, Ale Gru, Maxime Battistella, Rein Willemze and Youn Kim. On Behalf of the Cutaneous Lymphoma international Consortium (CLIC)

Introduction

Mycosis fungoides (MF) is the most prevalent form of primary cutaneous T-cell lymphoma. Patients frequently present with early-stage disease typically associated with a favourable prognosis, but over 25% may progress to advanced disease and 30% present with the advanced stages with a median survival < 4 years and of just 13 months in those with lymphomatous nodal effacement. An international study from 2015 in advanced MF found age >60 years, large cell transformation in skin, stage IV and raised LDH all to be associated with a worse prognosis and developed the CLIC prognostic index which stratified patients into low (0-1 factor) medium (2 factors) or high risk (3-4 factors) for poor survival [1]. In order to prospectively investigate prognostic factors in mycosis fungoides and Sezary syndrome the PROCLIPI International Study was launched in 2015 collecting predefined data [2].

Methods

We tested the prognostic index developed on a retrospective group of advanced MF/SS patients on a prospective group of advanced MF/SS patients from the PROCLIPI study.

Results

In PROCLIPI 1862 patients have been recruited at 51 sites, from 20 countries across 6 continents. 1442 early-stage patients (male:female ratio=1.7:1) and 420 advanced (male;female ratio 1.7:1). 297 patients from the advanced cohort had sufficient data to stratify according to the CLIC Prognostic Index to include n=112 low, n=118 medium and n=67 high risk patients. Applying this index found that low risk advanced MF/SS patients had a significantly better prognosis then high risk advanced patients p<0.001 as did the medium risk versus high risk p=0.005. Each group contained a variety of stages and 30% of the high risk patients were stages IIB-IVA1. This is important as comparing median survival between stages IIB, IIIA, IIIB and IVA1 did not identify any significant difference although the survival of this group versus stage IVA2 patients was significantly better (p=0.002).

Conclusions

Identifying prognostic factors at diagnosis may allow better management and improve survival and the development of a prognostic index could help identify patients at risk of progression.

References:

1. 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
Julia J Scarisbrick et al

2. The PROCLIPI international registry of early-stage mycosis fungoides identifies substantial diagnostic delay in most patients. Scarisbrick JJ, Br J Dermatol. 2019 Aug;181(2):350-357. doi