LY05 The plaque thickens: predictors of progression and prognosis in early-stage cutaneous T-cell lymphoma
Julia Scarisbrick, Abraham Bashir, Sean Whittaker, Christiane Querfeld, Maarten Vermeer, Evangelia Papadavid, Emilia Hodak, Martine Bagot, Pietro Quaglino, Octavio Servitje, Egle Ramelyte, Jose Sanches, Henry M Prince, Richard Cowan, Rudolf Stadler, Emmanuella Guenova, Anne-Marie Busschots, Oleg Akilov, Ulrike Wehkemp, Teresa Estrach, Sophie Weatherhead, Emilio Berti, Aikaterini Patsatsi, Annamari Ranki, Constanze Jonak, Pablo Ortiz-Romero, Marta Marschalko, Rubeta N Matin, Franz Trautinger, Giles Dunnill, Claus-Detlev Klemke, Mike Bayne, Cesare Massone, Ramon Pujol, Rachel Wachsmuth, Stephanie Schulz, Luke Bennett, Paula Enz, Marion Wobser, Larisa Geskin, Marie Beylot-Barry, Jan P Nicolay, Mona A Ibrahim, Arvind Aruimainathan, Pam Mackay, Jess Bailey, Deborah Turner, Pier Luigi Zinzani, Nicola Pimpinelli, Felicity Evison, Werner Kempf, Lorenzo Cerroni, Rein Willemze, Youn KimAbstract
The PROspective Cutaneous Lymphoma International Prognostic Index Study ‘PROCLIPI’ (ClinicalTrials.Gov ID: NCT02848274) opened in 2015 at > 50 international expert centres for mycosis fungoides (MF) and Sézary syndrome (SS). The aim of the study was to determine a prognostic index to better stratify patients for survival. A prognostic index for advanced MF and SS has recently been published that stratifies patients with advanced MF/SS into risk groups with significantly different 5-year overall survival. A CLIPI for early-stage MF is urgently needed to allow dermatologists to identify patients at risk of progression to advanced stage and subsequently select treatments for improved survival, as PROCLIPI shows that 5-year overall survival varies between 72.4% and 95.4% in early-stage MF and SS. Prospectively collected predefined datasets were analysed, including clinical, pathological, genotypic, treatment and quality-of-life data, in patients with newly diagnosed MF or SS. In total, 2004 patients with early-stage disease (IA, n = 921; IB, n = 853; IIA, n = 154) were recruited across 52 sites, presenting at a median age of 57 years (interquartile range 43–68). In multivariate analysis, the presence of cutaneous plaques (P < 0.001), nodal enlargement (Nx–N2) (P < 0.001), age > 60 years (P = 0.02) and large cell transformation in skin (P < 0.001) were significant factors for progression and overall survival. Notably, plaques have a high correlation with disease progression and poor overall survival (83.2% 5-year survival) compared with patients with patch-only disease (94.9% 5-year survival). Early-stage MF is typically reported as a low-grade lymphoma, but data from PROCLIPI have found low 5-year survival rates coupled with a median age of diagnosis of 57 years. Thus there is a marked reduction in life expectancy for some patients. However, 5-year survival rates for patients with patch-only disease are comparable with those of the average 57-year-old individual in Europe. In addition to plaques, PROCLIPI has identified other significant factors associated with poor survival, enabling the identification of at-risk patients using markers such as nodal enlargement and large cell transformation in the skin. The data highlight the need for better stratification of patients with early-stage MF and SS to allow improved management.