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Background: Total skin electron beam therapy (TSEBT) combined with systemic therapy or maintenance treatment is a reasonable approach to enhance the remission rate and duration in mycosis fungoides (MF) and Sézary syndrome (SS). This study assesses the efficacy of oral bexarotene therapy after low-dose TSEBT for patients with MF and SS., Methods: In this prospective observational study, we recruited MF/SS patients for treatment with low-dose total skin electron beam therapy (TSEBT) with or without bexarotene therapy to describe outcomes and toxicities., Results: Forty-six subjects with MF or SS underwent TSEBT between 2016 and 2021 at our institute. Following TSEBT, 27 patients (59 %) received oral bexarotene treatment. The median follow-up was 13 months. The overall response rate (ORR) for the cohort was 85 %. The response rate was significantly higher with combined modality (CM) than TSEBT alone (96 % vs. 68 %, p = 0.03). Median progression-free survival (PFS) for the CM was 17 months versus five months following TSEBT alone (p = 0.001). One patient (4 %) in the retinoid group discontinued the bexarotene therapy because of adverse events. The administration of bexarotene therapy did not increase radiation-related toxicities., Conclusions: Response rate and progression-free survival might be improved with TSEBT in combination with oral bexarotene compared to TSEBT alone., (© 2022 The Authors. Journal der Deutschen Dermatologischen Gesellschaft published by John Wiley & Sons Ltd on behalf of Deutsche Dermatologische Gesellschaft.)

Radiotherapy, particularly total skin electron beam therapy (TSEB), is one of the main pillars in the strategy for treatment of cutaneous T-cell lymphoma (CTCL). Low-dose TSEB has gained considerable attention since it has a minimal toxicity profile. Low-dose TSEB has been shown to yield an overall response rate up to 95 %, although the response duration is usually short. Few studies have been published on treatment outcomes after combined treatment of CTCL with TSEB and systemic therapy. Remission rates of patients who received immune checkpoint inhibitors alone ranged from 15-38 % with a two-year progression-free survival of 69 %. Given that TSEB results in rapid reduction of the disease burden in almost all patients, we hypothesized that TSEB followed by immune checkpoint inhibitors might be a reasonable treatment with a sustained effect for treatment-experienced patients with mycosis fungoides and Sezary syndrome., (© 2020 Deutsche Dermatologische Gesellschaft (DDG). Published by John Wiley & Sons Ltd.)