Global Patterns of Care in Advanced Stage Mycosis Fungoides/Sezary Syndrome: A Multicenter Retrospective Follow-Up Study from the Cutaneous Lymphoma International Consortium

Abstract

BACKGROUND: Advanced-stage Mycosis fungoides (MF)/Sézary syndrome (SS) patients are weighted by an unfavorable prognosis and share an unmet clinical need of effective treatments. International guidelines are available detailing treatment options for the different stages but without recommending treatments in any particular order due to lack of comparative trials. The aims of this second CLIC study were to retrospectively analyse the pattern of care world-wide for advanced-stage MF/SS patients, the distribution of treatments according to geographical areas (USA vs non-USA) and whether the heterogeneity of approaches have potential impact on survival.PATIENTS AND METHODS: This study included 853 patients from 21 specialist centres (14 European, 4 USA, 1 each Australian, Brazilian and Japanese).RESULTS: Heterogeneity of treatment approaches was found, with up to 24 different modalities or combinations used as first-line and 36% of patients receiving 4 or more treatments. Stage IIB disease was most frequently treated by total-skin-electron-beam radiotherapy, bexarotene and gemcitabine; erythrodermic and SS patients by extracorporeal photochemotherapy, and stage IVA2 by polychemotherapy. Significant differences were found between USA and non-USA centres, with bexarotene, photopheresis and histone deacetylase inhibitors most frequently prescribed for first line treatment in USA whilst phototherapy, interferon, chlorambucil and gemcitabine in non-USA centres. These differences did not significantly impact on survival. However, when considering death and therapy change as competing risk events and the impact of first treatment line on both events, both monochemotherapy (SHR=2.07) and polychemotherapy (SHR=1.69) showed elevated relative risks.CONCLUSION: this large multicentre retrospective study shows there exist a large treatment heterogeneity in advanced MF/SS and differences between USA and non-USA centres but these were not related to survival, whilst our data reveal that chemotherapy as first treatment is associated with a higher risk of death and/or change of therapy and thus other therapeutic options should be preferable as first treatment approach.

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