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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 05/22/2008



Purpose of This PDQ Summary






General Information






Cellular Classification






Stage Information






Treatment Option Overview






Stage I Mycosis Fungoides/Sézary Syndrome






Stage II Mycosis Fungoides/Sézary Syndrome






Stage III Mycosis Fungoides/Sézary Syndrome






Stage IV Mycosis Fungoides/Sézary Syndrome






Recurrent Mycosis Fungoides/Sézary Syndrome






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Changes to This Summary (05/22/2008)






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Recurrent Mycosis Fungoides/Sézary Syndrome

Current Clinical Trials

The treatment of relapsed patients with cutaneous T-cell lymphomas involves the joint decisions of the dermatologist, medical oncologist, and radiation oncologist. It may be possible to re-treat localized areas of relapse with additional electron-beam radiation or possibly to repeat total-skin electron-beam radiation therapy.[1] Photon radiation to bulky skin or nodal masses may prove beneficial. If these options are not possible, then continued topical treatment with other modalities such as mechlorethamine or psoralen and ultraviolet A radiation (PUVA) may be warranted to relieve cutaneous symptoms.

Clinical trials, if possible, should be considered as the next therapeutic option. Options include:[2]

  • PUVA combined with interferon-alpha-2a is associated with a high response rate.[3]
  • Extracorporeal photochemotherapy has produced tumor regression in patients resistant to other therapies.[4,5]
  • Radioimmunotherapy using an I-131-labeled monoclonal antibody directed against a T-cell antigen has produced brief responses in a clinical trial.[6]
  • The interleukin-2 fusion toxin, denileukin diftitox, is given monthly with response rates of about 30% to 40% for patients with CD25 and mycosis fungoides (MF).[7]
  • Bexarotene is a retinoid available in an oral or topical form with activity in patients with recurrent MF, as evidenced in the L-1069 trial.[8,9]
  • New purine nucleoside phosphorylase inhibitors such as peldesine are under clinical evaluation.[10]
  • Allogeneic or autologous bone marrow transplantation is also under clinical evaluation.[11]
Current Clinical Trials

Check for U.S. clinical trials from NCI's PDQ Cancer Clinical Trials Registry that are now accepting patients with recurrent mycosis fungoides/Sezary syndrome. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

General information about clinical trials is also available from the NCI Web site.

References

  1. Becker M, Hoppe RT, Knox SJ: Multiple courses of high-dose total skin electron beam therapy in the management of mycosis fungoides. Int J Radiat Oncol Biol Phys 32 (5): 1445-9, 1995.  [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. 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]

  4. Edelson R, Berger C, Gasparro F, et al.: Treatment of cutaneous T-cell lymphoma by extracorporeal photochemotherapy. Preliminary results. N Engl J Med 316 (6): 297-303, 1987.  [PUBMED Abstract]

  5. Heald PW, Perez MI, McKiernan G, et al.: Extracorporeal photochemotherapy for CTCL. Prog Clin Biol Res 337: 443-7, 1990.  [PUBMED Abstract]

  6. 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]

  7. 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]

  8. Miller VA, Benedetti FM, Rigas JR, et al.: Initial clinical trial of a selective retinoid X receptor ligand, LGD1069. J Clin Oncol 15 (2): 790-5, 1997.  [PUBMED Abstract]

  9. 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]

  10. Henderson B, Poole S, Wilson M: Microbial/host interactions in health and disease: who controls the cytokine network? Immunopharmacology 35 (1): 1-21, 1996.  [PUBMED Abstract]

  11. Molina A, Zain J, Arber DA, et al.: Durable clinical, cytogenetic, and molecular remissions after allogeneic hematopoietic cell transplantation for refractory Sezary syndrome and mycosis fungoides. J Clin Oncol 23 (25): 6163-71, 2005.  [PUBMED Abstract]

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