Systemic Treatment Options for Advanced-Stage Mycosis Fungoides and Sézary Syndrome

Curr Oncol Rep. 2018 Mar 23;20(4):32. doi: 10.1007/s11912-018-0678-x.

Abstract

Purpose of review: Cutaneous T-cell lymphoma (CTCL) is a rare form of non-Hodgkin lymphoma. Globally, the most common subtypes of CTCL are mycosis fungoides and Sézary syndrome. CTCL can confer significant morbidity and even mortality in advanced disease. Here we review the current and potential future treatments for advanced-stage CTCL.

Recent findings: Heterogeneity of treatment choice has been demonstrated both in US and non-US centers. Systemic treatment choice is currently guided by prognostic features, incorporating stage, immunophenotypic and molecular findings, and patient-specific factors such as age and comorbidities. Randomized controlled studies are uncommon, and the literature is composed predominantly of retrospective, cohort, and early-phase studies. International consensus guidelines are available; however, the lack of comparative trials means that there is no clear algorithmic approach to treatment. This review article reports on the systemic treatment options in current use for advanced CTCL, and on the possible future therapies, acknowledging that an algorithmic approach is not yet forthcoming to guide treatment prioritization.

Keywords: Allogeneic stem cell transplant; Bendamustine; Bexarotene; Brentuximab vedotin; Cutaneous T-cell lymphoma; Denileukin diftitox; Doxorubicin; Extracorporeal photopheresis; Gemcitabine; Histone deacetylase inhibitors; Interferon-alfa; Methotrexate; Mogamulizumab; Monoclonal antibodies; Mycosis fungoides; Pentostatin; Pralatrexate; Romidepsin; Sézary syndrome; Vorinostat.

Publication types

  • Review

MeSH terms

  • Combined Modality Therapy
  • Humans
  • Mycosis Fungoides / pathology
  • Mycosis Fungoides / therapy*
  • Prognosis
  • Sezary Syndrome / pathology
  • Sezary Syndrome / therapy*
  • Skin Neoplasms / pathology
  • Skin Neoplasms / therapy*