Prolonged survival with the early use of a novel extracorporeal photopheresis regimen in patients with Sézary Syndrome

Crystal Gao, Christopher McCormack, Carrie van der Weyden, Michelle S. Goh, Belinda Campbell, Robert Twigger, Odette Buelens, Simon J. Harrison, Christine Khoo, Stephen Lade and H. Miles Prince

Key Points

  • Use of extracorporeal photopheresis prolongs survival and disease control in patients with Sézary Syndrome/erythrodermic mycosis fungoides

  • Extracorporeal photopheresis should be commenced as early as possible in the treatment paradigm for MF/SS, ideally at treatment lines 1-3


Extracorporeal photopheresis (ECP) has demonstrated therapeutic benefit in patients with Sézary Syndrome (SS) and erythrodermic mycosis fungoides (MF). To examine the efficacy of ECP in the modern era of novel therapies, we conducted a retrospective analysis of 65 patients with a diagnosis of SS or erythrodermic MF with blood involvement who were treated with ECP at our institute. Overall survival (OS) and time-to-next-treatment (TTNT) were used as the study endpoints to determine patient outcome. The median follow-up from diagnosis was 48 months (range 1-225 months), with a median predicted OS of 120 months. The majority (88%) of patients commenced ECP at treatment lines 1-3, either as a monotherapy or in conjunction with other systemic agents. The use of ECP monotherapy resulted in a significantly longer TTNT when compared to: interferon alpha (p = 0.0067), histone deacetylase inhibitors (p = 0.0003), novel immunotherapy agents (p = 0.028), low dose methotrexate (p < 0.0001), and chemotherapy (p < 0.0001). In particular, early commencement of ECP at treatment lines 1-3 yielded a TTNT of 47 months. The results of our study support the utilization of ECP for SS/erythrodermic MF, and we recommend that ECP should be considered as early as possible in the treatment paradigm for these patients.

  • Submitted March 25, 2019.
  • Revision received August 28, 2019.
  • Accepted July 30, 2019.