What are the guidelines on the treatment of mycosis fungoides/Sezary syndrome (MF/SS)?

Updated: Feb 25, 2021
  • Author: Sanjay Vinjamaram, MD, MPH; Chief Editor: Emmanuel C Besa, MD  more...
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The NCCN recommends that patients be treated at specialized centers with expertise in the management of MF/SS. Unlike other non-Hodgkin lymphoma subtypes, MF/SS criteria have not correlated with prognosis, and treatment decisions are made on a clinical basis. [26]

In 2011, the International Society for Cutaneous Lymphomas, the United States Cutaneous Lymphoma Consortium, and the Cutaneous Lymphoma Task Force of the EORTC issued a joint consensus statement proposing new clinical end points and response criteria for use in clinical trials of MF/SS. [87]  The NCCN includes the new criteria in its current recommendations. [26]

All three guidelines recommend treatment selection based on clinical stage. [26, 84, 85]

Mycosis fungoides

In general, topical therapies are recommended for localized disease (stages IA, IB and IIA), with systemic or combination systemic and topical therapies beginning with stage IIB. Systemic therapies may also be considered in patients with early-stage disease that does not respond to topical treatments. [26, 84, 85]

Localized therapies endorsed by the guidelines include the following [26, 84, 85]

  • Psoralens + ultraviolet A (PUVA)—For thick plaques

  • Narrow-band ultraviolet B (UVB)—For patch or thin plaques

  • Topical corticosteroids

  • Localized radiation therapy (12-36 Gy)

  • Topical chemotherapy (ie, nitrogen mustard or carmustine)

  • Topical retinoids (ie, bexarotene)

Because of the high toxicity of total skin electron beam therapy (TSEBT), both NCCN and EOTRTC recommend TSEBT only after other treatments have failed. [26, 84]

Systemic therapies recommended for advanced stages include the following [26, 84, 85]

  • Retinoids (ie, bexarotene)

  • Interferons (IFN-alpha, IFN-gamma)

  • Histone deacetylase (HDAC)-inhibitors (vorinostat, romidenpsin)—not approved in Europe

  • Extracorporeal photopheresis (ECP)

  • Methotrexate (≤100 mg/wk)

All three guidelines recommend IFN-alpha and PUVA or PUVA and retinoids (including bexarotene) as second-line combination therapies for MF stages IA, IB, and IIA. [26, 84, 85]

Other NCCN recommended combination therapies for advanced disease include the following [26] :

  • PUVA and ECP

  • TSEBT and ECP

  • Retinoids and interferons

  • ECP and retinoids

  • ECP and interferons

  • ECP and retinoids and interferons

Sezary syndrome and erythrodermic mycosis fungoides

Participation in a clinical trial as a treatment option for all patients with SS or advanced-stage MF is recommended by both the NCCN and EOTRTC.  [26, 84]  Treatment for SS or erythrodermic MF (stage IV) is palliative. The preferred modality is ECP, either alone or in combination with other treatments. [26, 84, 85]

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