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Current systemic therapeutic options for advanced mycosis fungoides and Sézary syndrome

Jenna Janiga1, Jonathan Kentley2, Chadi Nabhan3

  • 1a Stritch School of Medicine , Loyola University , Chicago , IL , USA.

Leukemia & Lymphoma
|January 9, 2018
PubMed

Insights

Current treatments for advanced cutaneous T-cell lymphomas like mycosis fungoides and Sézary syndrome offer limited durable remission. Research into new therapies is ongoing, but standard options remain non-curative with low response rates.

Area of Science:

  • Oncology
  • Dermatology
  • Immunology

Background:

  • Mycosis fungoides (MF) and Sézary syndrome (SS) are the most prevalent cutaneous T-cell lymphomas (CTCLs).
  • Advanced-stage CTCLs lack curative treatments and are associated with a poor prognosis.
  • Achieving durable remission with current therapies remains a significant clinical challenge.

Purpose of the Study:

  • To review current systemic monotherapy options for advanced-stage MF (IIB-IV).
  • To analyze the efficacy, mechanism of action, and toxicities of existing treatments.
  • To highlight the limitations of current standard of care in achieving durable responses.

Main Methods:

  • Literature review of systemic monotherapy for advanced-stage MF.
  • Appraisal of treatment mechanisms, efficacy (overall response rates - ORR), and toxicity profiles.
  • Analysis of reported response durations, typically ranging from 7.5 to 22.4 months.

Main Results:

  • Reported ORR for individual therapies vary widely across studies.
  • Duration of responses to current monotherapies is generally short.
  • Combined therapies are used but lack robust comparative prospective studies against monotherapy.

Conclusions:

  • Current standard of care therapies for advanced-stage MF and SS are not curative.
  • Despite advances in understanding immunopathogenesis, existing treatments have low ORR and limited durability.
  • There is a critical need for novel, curative therapeutic strategies for advanced CTCLs.
Keywords:
CTCLMycosis fungoidesSézary syndromemanagementtreatment

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