Clinical severity measures and quality-of-life burden in patients with mycosis fungoides and Sezary syndrome: comparison of generic and dermatology-specific instruments

Background Given the severe symptom burden and chronic nature of mycosis fungoides (MF) and Sezary syndrome (SS), effective assessment of quality of life (QoL) is essential to guiding patient-centred care in this population. In this study, we aim to provide a comprehensive assessment of QoL in early- and advanced-stage MF/SS and to assess the correlation of traditional measures of clinical severity with QoL measures. Methods Between July 2017 and April 2019, outpatients at an academic medical centre with either MF/SS (n = 115) or general dermatology concerns (n = 115) completed generic and dermatology-specific QoL instruments [Health Utilities Index Mark 3 (HUI3), RAND 36-Item Short-Form Health Survey (SF-36), Skindex-29, visual analogue scale for itch (VAS itch) and 5-D pruritus scale]. The mean scores of MF/SS patients were compared to that of controls using multivariable regression models adjusted for demographics and medical comorbidities. Cluster analysis of the QoL instruments and clinical severity measures (e.g. stage and body-surface-area involvement) was performed. Results Patients with MF/SS scored significantly worse than controls on all QoL instruments used, with advanced-stage (IIB-IVB) disease having the worst QoL impairment. Early-stage (IA-IIA) and advanced-stage MF/SS patients had significantly reduced overall health status (HUI3; P < 0.05), with largest decrements in social functioning and usual role functioning due to physical and emotional health (SF-36; all P < 0.05). MF/SS had significantly worse skin-specific impairment than controls, with advanced-stage disease reporting the most severe skin-specific burden (Skindex-29, P < 0.05). Clinical severity measures had a weak correlation with generic (|r(s)| = 0.02-0.27) and moderate correlation with dermatology-specific instruments (|r(s)| = 0.41-0.53). Conclusions MF/SS have a significant impact on multiple domains of patients' QoL, including social, emotional and physical functioning. Current clinical measures do not adequately address QoL outcomes, underscoring the need for integrating formal disease-specific QoL assessment into the routine evaluation of MF/SS patients.