Maintenance therapy in patients with mycosis fungoides or Sézary syndrome: A neglected topic

Abstract

In advanced-stage cutaneous T-cell lymphoma (CTCL), the current therapeutic options rarely provide long-lasting responses, leaving allogenic stem-cell transplantation the only potentially curative option for highly selected patients. Until today, there are no standardised strategies for maintenance therapy in patients with CTCL who achieved disease control. Moreover, evidence-based treatment options or drugs that are approved for maintenance treatment in advanced stages after remission induction are still lacking. Patients require maintenance options that provide a good safety profile, are convenient to apply and do not negatively affect their health-related quality of life. However, carrying out large-scale, controlled studies is challenging in CTCL. In addition to information on the concept of maintenance therapy, this review provides an update on current and emerging approaches that target maintenance treatment in advanced-stage CTCL. After all, the group of potentially interesting maintenance therapy candidates, especially for patients in advanced stage, includes not only immunomodulating and phototherapeutic modalities that have been used already for many decades but also newer systemic therapies, including epigenetic modifiers.

Keywords: Cutaneous T-cell lymphoma; HDAC inhibitors; Maintenance therapy.