What systemic treatments are used in the management of cutaneous T-cell lymphoma (CTCL)?

Updated: Aug 15, 2018
  • Author: Lauren C Pinter-Brown, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Systemic treatment, such as oral retinoids, recombinant interferon alfa, fusion toxins, monoclonal antibodies, and single-agent chemotherapy, can be used sequentially to palliate symptoms from more advanced mycosis fungoides. Many active agents exist, including antimetabolites, alkylating agents, topoisomerase II inhibitors, anthracyclines, and purine analogues. The most extensive data exist for the use of methotrexate. [104]

Other examples of systemic treatment for mycosis fungoides include the following:

  • Extracorporeal photopheresis - Leukapheresis with UV-A light treatment enhanced with psoralen (PUVA) for the collected white blood cells, with reinfusion of treated cells

  • Oral retinoids

  • Fusion toxin treatment - Recombinant toxins are generated by fusion of a plant or bacterial toxin gene to a receptor ligand; the first such toxin to enter clinical trial was DAB-interleukin 2 (DAB-IL2)

  • Histone deacetylase inhibitor treatment - These agents may inhibit gene transcription

  • CC chemokine receptor type 4 (CCR4)–directed monoclonal antibody

Biologic response modifiers may inhibit the proliferative capacity of malignant T cells by modulating cytokine production. These agents may also enhance the antitumor immune response by augmenting cell-mediated cytotoxicity.

Mogamulizumab, a CCR4-directed monoclonal antibody, was approved by the FDA in August 2018 for adults with mycosis fungoides or Sézary syndrome who have received at least 1 prior systemic therapy. Approval was based on the MAVORIC (Mogamulizumab anti-CCR4 Antibody Versus ComparatOR In CTCL) phase 3 clinical trial that compared mogamulizumab with vorinostat. Significantly superior progression-free survival (PFS) was observed with mogamulizumab (7.7 months) compared with vorinostat (3.1 months) (P < 0.0001). [105]


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