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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®)

Health Professional Version

Changes to This Summary (01/24/2014)

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 and Sézary Syndrome

Added Agar et al., Talpur et al., and Kim et al., as references 5, 6, and 7, respectively.

Added text to state that a retrospective study with a median follow-up of 14.5 years showed that 20% of 1,422 patients progressed from stage I or II disease to stage II or IV disease. Also added that the same retrospective study found that only 3% of the patients progressed from mycosis fungoides to the Sézary syndrome (cited Quaglino et al. as reference 14). Also revised text to state that cytologic transformation from a low-grade lymphoma to a high-grade lymphoma (large-cell transformation) occurs rarely (<5%) during the course of these diseases and is associated with a poor prognosis.

Stage Information for Mycosis Fungoides and the Sézary Syndrome

Added Agar et al. as reference 2.

Treatment Option Overview

Revised text in the treatment option list to read vorinostat or romidepsin or other histone deacetylase inhibitors.

Stage I Mycosis Fungoides

Added Lessin et al. and Thomas et al. as references 13 and 15, respectively. Also revised text in treatment option 11 to read vorinostat or romidepsin or other histone deacetylase inhibitors (cited Duvic et al. as reference 20). Added Prince et al. as reference 24.

Stage II Mycosis Fungoides

Added Lessin et al., Thomas et al., and Dummer et al. as references 10, 12, and 17, respectively. Also revised text in treatment option 11 to read vorinostat or romidepsin or other histone deacetylase inhibitors (cited Duvic et al. as reference 20). Added Prince et al. as reference 24.

Stage III Mycosis Fungoides

Added Thomas et al., Prince et al., Lessin et al., and Dummer et al. as references 8, 14, 22, and 27, respectively. Also revised text in treatment option 15 to read vorinostat or romidepsin or other histone deacetylase inhibitors (cited Duvic et al. as reference 30).

Stage IV Mycosis Fungoides

Added Prince et al., Lessin et al., and Dummer et al. as references 13, 17, and 29, respectively. Also revised text in treatment option 16 to read vorinostat or romidepsin or other histone deacetylase inhibitors (cited Duvic et al. as reference 32).

Recurrent Mycosis Fungoides and the Sézary Syndrome

Added Prince et al. as reference 8. Also revised text to read vorinostat or romidepsin or other oral histone deacetylase inhibitors (cited Duvic et al. as reference 16).

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.

  • Updated: January 24, 2014