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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)

  • Last Modified: 01/24/2014

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Treatment Option Overview

Treatment options for patients with mycosis fungoides and the Sézary syndrome (MF/SS) include:[1-3]

  • Topical corticosteroids.
  • Topical chemotherapy with mechlorethamine (nitrogen mustard) or carmustine (BCNU).
  • Psoralen and ultraviolet A radiation (PUVA).
  • Ultraviolet B radiation (UVB).
  • Total-skin electron-beam radiation (TSEB).
  • Radiation of symptomatic skin lesions.
  • Interferon-alpha or interferon-gamma alone or in combination with topical therapy.
  • Single-agent and multiagent chemotherapy.
  • Bexarotene (topical gel or oral); retinoids.
  • Denileukin diftitox (recombinant fusion protein of diphtheria toxin fragments and interleukin-2 sequences).
  • Vorinostat or romidepsin or other histone deacetylase inhibitors.
  • Pralatrexate (folate analog).[4]
  • Alemtuzumab (a humanized monoclonal antibody targeting the CD52 antigen).
  • Combined modality treatment.

These types of treatments produce remissions, but long-term remissions are uncommon. Treatment, therefore, is considered palliative for most patients, though major symptomatic improvement is regularly achieved. Survival in excess of 8 years, however, is common for patients with early stages of disease. All patients with MF/SS are candidates for clinical trials evaluating new approaches to treatment.

Current areas of interest in clinical trials for MF confined to the skin include combined modality therapies containing both topical and systemic agents such as TSEB combined with chemotherapy, topical mechlorethamine or PUVA combined with interferon, or wide-field radiation techniques with PUVA. Reports are available of activity for extracorporeal photochemotherapy using psoralen; interferon-gamma or interferon-alpha; pentostatin; retinoids; fludarabine; acyclovir; 2-chlorodeoxyadenosine; serotherapy with unlabeled, toxin-labeled, or radiolabeled monoclonal antibodies; cell surface receptor ligand-toxin fusion protein; and, methotrexate.[3,5-14] Antigen-specific vaccination using dendritic cells [15] and UVB are also under clinical evaluation.

References
  1. Prince HM, Whittaker S, Hoppe RT: How I treat mycosis fungoides and Sézary syndrome. Blood 114 (20): 4337-53, 2009.  [PUBMED Abstract]

  2. Trautinger F, Knobler R, Willemze R, et al.: EORTC consensus recommendations for the treatment of mycosis fungoides/Sézary syndrome. Eur J Cancer 42 (8): 1014-30, 2006.  [PUBMED Abstract]

  3. Olsen EA, Rook AH, Zic J, et al.: Sézary syndrome: immunopathogenesis, literature review of therapeutic options, and recommendations for therapy by the United States Cutaneous Lymphoma Consortium (USCLC). J Am Acad Dermatol 64 (2): 352-404, 2011.  [PUBMED Abstract]

  4. Horwitz SM, Kim YH, Foss F, et al.: Identification of an active, well-tolerated dose of pralatrexate in patients with relapsed or refractory cutaneous T-cell lymphoma. Blood 119 (18): 4115-22, 2012.  [PUBMED Abstract]

  5. Kaplan EH, Rosen ST, Norris DB, et al.: Phase II study of recombinant human interferon gamma for treatment of cutaneous T-cell lymphoma. J Natl Cancer Inst 82 (3): 208-12, 1990.  [PUBMED Abstract]

  6. Heald P, Rook A, Perez M, et al.: Treatment of erythrodermic cutaneous T-cell lymphoma with extracorporeal photochemotherapy. J Am Acad Dermatol 27 (3): 427-33, 1992.  [PUBMED Abstract]

  7. Rosen ST, Zimmer AM, Goldman-Leikin R, et al.: Radioimmunodetection and radioimmunotherapy of cutaneous T cell lymphomas using an 131I-labeled monoclonal antibody: an Illinois Cancer Council Study. J Clin Oncol 5 (4): 562-73, 1987.  [PUBMED Abstract]

  8. Foss FM, Ihde DC, Breneman DL, et al.: Phase II study of pentostatin and intermittent high-dose recombinant interferon alfa-2a in advanced mycosis fungoides/Sézary syndrome. J Clin Oncol 10 (12): 1907-13, 1992.  [PUBMED Abstract]

  9. Saven A, Carrera CJ, Carson DA, et al.: 2-Chlorodeoxyadenosine: an active agent in the treatment of cutaneous T-cell lymphoma. Blood 80 (3): 587-92, 1992.  [PUBMED Abstract]

  10. Kuzel TM, Roenigk HH Jr, Samuelson E, et al.: Effectiveness of interferon alfa-2a combined with phototherapy for mycosis fungoides and the Sézary syndrome. J Clin Oncol 13 (1): 257-63, 1995.  [PUBMED Abstract]

  11. Olsen E, Duvic M, Frankel A, et al.: Pivotal phase III trial of two dose levels of denileukin diftitox for the treatment of cutaneous T-cell lymphoma. J Clin Oncol 19 (2): 376-88, 2001.  [PUBMED Abstract]

  12. Siegel RS, Pandolfino T, Guitart J, et al.: Primary cutaneous T-cell lymphoma: review and current concepts. J Clin Oncol 18 (15): 2908-25, 2000.  [PUBMED Abstract]

  13. Duvic M, Hymes K, Heald P, et al.: Bexarotene is effective and safe for treatment of refractory advanced-stage cutaneous T-cell lymphoma: multinational phase II-III trial results. J Clin Oncol 19 (9): 2456-71, 2001.  [PUBMED Abstract]

  14. Zackheim HS, Kashani-Sabet M, McMillan A: Low-dose methotrexate to treat mycosis fungoides: a retrospective study in 69 patients. J Am Acad Dermatol 49 (5): 873-8, 2003.  [PUBMED Abstract]

  15. Maier T, Tun-Kyi A, Tassis A, et al.: Vaccination of patients with cutaneous T-cell lymphoma using intranodal injection of autologous tumor-lysate-pulsed dendritic cells. Blood 102 (7): 2338-44, 2003.  [PUBMED Abstract]