The changing therapeutic landscape, burden of disease, and unmet needs in patients with cutaneous T-cell lymphoma

Br J Haematol. 2021 Feb;192(4):683-696. doi: 10.1111/bjh.17117. Epub 2020 Oct 23.

Abstract

Cutaneous T-cell lymphomas (CTCLs) have a chronic, relapsing course, and the most common subtypes are mycosis fungoides and Sézary syndrome. The disease causes visible skin alterations and can also cause alopecia, pruritus and pain, all of which can impact patients' health-related quality of life (HRQoL). The goal of treatment is to reduce symptoms and prevent disease progression. However, treatment recommendations are often based on low levels of evidence due to the lack of well-designed randomised clinical trials and treatment guidelines, and approved drugs vary considerably across different countries and regions. Currently, available treatments rarely lead to durable remissions and eventually become less effective, meaning patients often require multiple therapy changes. Skin-directed therapies (SDTs) are first-line treatments for early-stage CTCL, whereas systemic therapies may be needed for early-stage disease that does not respond to SDT or for advanced-stage disease. However, patients can experience significant side-effects with these treatments or may be unable to tolerate them. Hence, there is an unmet need for effective therapies with good safety profiles for the treatment of early- and late-stage CTCL. Here, we review current treatment guidelines, investigational and approved treatments, the impact of CTCL on patients' HRQoL, and the treatment of pruritus.

Keywords: CTCL; Sézary syndrome; mycosis fungoides; quality of life; treatment strategies.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cost of Illness
  • Disease Management
  • Humans
  • Lymphoma, T-Cell, Cutaneous / economics
  • Lymphoma, T-Cell, Cutaneous / therapy*
  • Quality of Life
  • Skin Neoplasms / economics
  • Skin Neoplasms / therapy*

Grants and funding