Current systemic therapeutic options for advanced mycosis fungoides and Sézary syndrome

Abstract

Mycosis fungoides (MF) and Sézary syndrome (SS) are the most common cutaneous T-cell lymphomas (CTCLs). Both lack curative options, and advanced-stage carries a poor prognosis. Whilst there are a number of treatments available, achieving and maintaining a durable remission remains challenging. We review current systemic treatment options as monotherapy for advanced-stage MF (IIB–IV), appraising their mechanism of action, analyzing their efficacy, and describing toxicities. Individually, reported overall response rates (ORR) vary widely in the literature and duration of responses are typically short, ranging from 7.5 to 22.4 months. Combined therapy is frequently used in an effort to boost responses, although prospective studies comparing combinations to single agent therapies are rarely conducted. While recent translational research has led to increased understanding of the immunopathogenesis of MF and SS and the development of new treatments, current standard of care therapies are not curative and have low ORR for advanced-stage disease.

Keywords:

• Mycosis fungoides
• Sézary syndrome
• CTCL
• management
• treatment

Potential conflict of interest

Disclosure forms provided by the authors are available with the full text of this article online at https://doi.org/10.1080/10428194.2017.1347650.