Mycosis Fungoides (Including Sézary Syndrome) Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (07/12/2017)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

General Information About Mycosis Fungoides (MF) (Including Sézary Syndrome [SS])

Revised text to state that the cutaneous T-cell lymphomas should be distinguished from other T-cell lymphomas that involve the skin, such as anaplastic large cell lymphoma, peripheral T-cell lymphoma, adult T-cell leukemia/lymphoma, or subcutaneous panniculitic T-cell lymphoma.

Added Alberti-Violetti et al. as reference 9. Added text to state that the Cutaneous Lymphoma International Consortium retrospectively reviewed 1,275 patients and found four independent prognostic markers that indicate a worse survival, including stage IV disease, age older than 60 years, large cell transformation, and elevated lactate dehydrogenase (cited Scarisbrick et al. as reference 10).

Added R. Volmer as reference 12. Revised text to state that in contrast, more than 50% of patients with stage III through stage IV disease die of MF, with a median survival of approximately 5 years. Also added that the Cutaneous Lymphoma International Prognostic index used male gender, age older than 60 years, plaques, lymph nodes, blood involvement, and visceral involvement as poor prognostic factors to define predicted overall survival (OS) and progression-free survival in both early-stage groups and advanced-stage groups (cited Benton et al. as reference 15).

Revised text to state that cutaneous disease can manifest itself as an eczematous patch or plaque stage covering less than 10% of the body surface, a plaque stage covering 10% or more of the body surface, or as tumors that frequently undergo necrotic ulceration. Also revised the text about several retrospective studies, which showed that 20% of patients progressed from stage I or II disease to stage III or IV disease (cited Wernham et al. as reference 19 and Desai et al. as reference 20).

Treatment Option Overview for MF (Including SS)

Added Olsen et al. as reference 4.

Added text to state that total-skin electron-beam radiation therapy can provide excellent palliation, with complete response rates of as much as 80%, and may be combined with systemic treatment. Also added ultraviolet B radiation or ultraviolet A radiation as radiation therapy options.

Added Targeted Therapy as a new subsection.

Added text to state that among these highly selected patients, the 5-year OS rate ranges from 30% to 50%, with a relapse-free survival rate of 15% to 25% (cited Lechowicz et al. as reference 50).

Stage I and Stage II MF

This section was extensively revised.

Stage III and Stage IV MF (Including SS)

This section was extensively revised.

Recurrent MF (Including SS)

Revised text to include allogeneic bone marrow or stem cell transplantation for the transplantation treatment option under clinical evaluation (cited Lechowich et al. as reference 30).

Added text to include brentuximab vedotin, a targeted therapy, as a treatment option under clinical evaluation (cited Kim et al. as reference 31 and Duvic et al. as reference 32).

Key References for MF (Including SS)Treatment

Editorial changes were made to this section.

This summary is written and maintained by the PDQ Adult Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ® - NCI's Comprehensive Cancer Database pages.

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