The Plaque Thickens: Predictors of Progression and Prognosis in Early-Stage Cutaneous T-cell Lymphoma

Abstract 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.