Prognostic factors for early and advanced stage MF/SS

The stage of mycosis fungoides (MF) and Sézary syndrome (SS) do not predict survival well. A prognostic index stratifies advanced patients into low, intermediate, and high-risk for worse survival. These were the main takeaways from the PROCLIPI (Prospective Cutaneous Lymphoma International Prognostic Index) study, which also identified prognostic factors for early- and for advanced-stage disease.

Since 2015, PROCLIP has recruited 2,469 patients at 52 expert MF/SS centres from 19 countries. Prof. Julia Scarisbrick (University Hospital Birmingham, UK) presented early-stage data of 1,794 patients (77%), and late-stage data of 534 patients (24%) [1]. Median age at stage IA was significantly lower than at stage IB (57 vs 60 years; P=0.032). Median age at early-stage disease (IA-IIA) was also lower than at late-stage disease (IIB-IVB: 58 versus 67 years (P<0.0001). Patients presenting with advanced disease had a relatively short disease duration before diagnosis.

Significant risk factors for progression of patients presenting in an early stage were: nodule enlargement (N1: HR 3.89; P=0.001; Nx: HR 2.44; P<0.001), age >60 years (HR 2.08; P<0.001), plaques (HR 3.39; P<0.001), and large cell transformation (LCT) in the skin (HR 4.73; P<0.001).

The 5-year overall survival (OS) rates in the early cohort (n=1,581) were: 94.8% for stage IA, 83.7% for stage IB, and 73.4% for stage IIA. The corresponding rates in the advanced cohort (n=533) were: 51.11% for stage IIB, 62.3% for stage IIIA, 49.4% for stage IIIB, 50.7% for stage IVA1, 22.8% for stage IVA2, and 36.9% for stage IVB. “Staging does not predict survival,” Prof. Scarisbrick observed.

Factors at diagnosis significantly associated with poor survival in advanced stages were: N3 status (P<0.001), age >60 years (P=0.001), raised serum LDH above normal (P=0.003), and LCT in skin (P=0.073). These 4 independent risk factors were modelled into a prognostic index with a low-, an intermediate-, and a high-risk group, each one predicting a significantly different OS versus the other 2 risk factors.

1. Scarisbrick, et al. Identifying patients with poor outcomes in mycosis fungoides & Sezary syndrome for improved management choices. Abstract A-162, EORTC-CLTG 2024, 9-11 October 2024, Lausanne, Switserland.

Posted on 11 October 202411 October 2024