From the Guidelines
Treatment for mycosis fungoides should be stage-based, with skin-directed therapies as the mainstay for early-stage disease (IA-IIA) and systemic therapies for more advanced stages (IIB-IV). The most recent and highest quality study 1 provides guidance on the management of primary cutaneous lymphomas, including mycosis fungoides.
Key Treatment Options
- For early-stage disease (IA-IIA), skin-directed therapies are recommended, including:
- Topical corticosteroids (betamethasone, clobetasol)
- Topical retinoids (bexarotene gel)
- Topical chemotherapy (nitrogen mustard, carmustine)
- Phototherapy (narrowband UVB for patch stage, PUVA for plaque stage)
- Localized radiation therapy
- For more advanced stages (IIB-IV), systemic therapies become necessary, including:
- Retinoids (bexarotene 300 mg/m² daily)
- Interferon alfa (3-10 million units three times weekly)
- Histone deacetylase inhibitors (vorinostat 400 mg daily, romidepsin 14 mg/m² on days 1,8, and 15 of a 28-day cycle)
- Monoclonal antibodies (mogamulizumab, brentuximab vedotin)
- Traditional chemotherapy
Additional Considerations
- Allogeneic stem cell transplantation may be considered for younger patients with advanced, refractory disease 1.
- Treatment plans should be adjusted based on response and tolerability, with regular skin examinations and imaging studies to monitor disease status 1.
- Combination therapies are often used, and the goal of treatment is to control symptoms and disease progression rather than cure, as mycosis fungoides is generally chronic 1.
From the FDA Drug Label
1.7 Relapsed Primary Cutaneous Anaplastic Large Cell Lymphoma (pcALCL) or CD30-Expressing Mycosis Fungoides (MF) ADCETRIS is indicated for the treatment of adult patients with pcALCL or CD30-expressing MF who have received prior systemic therapy.
Treatment options for Mycosis Fungoides include brentuximab vedotin (IV) for adult patients with CD30-expressing Mycosis Fungoides (MF) who have received prior systemic therapy 2.
- Key points:
- The patient must have CD30-expressing MF.
- The patient must have received prior systemic therapy.
- Brentuximab vedotin (IV) is indicated for the treatment of adult patients with this condition.
From the Research
Treatment Options for Mycosis Fungoides
The treatment of mycosis fungoides (MF) aims to reduce symptoms, improve clinical appearance, prevent secondary complications, and prevent progression of disease, all of which may have an impact on survival 3.
- Topical and Systemic Therapies: Treatment of MF includes topical and systemic therapies, which can be administered alone or in combination 3.
- Psoralen and Ultraviolet A Radiation: Psoralen and ultraviolet A radiation is effective in early-stage MF, inducing complete remissions in most patients 3.
- Narrowband UVB Phototherapy: Narrowband UVB (NB-UVB) therapy has been shown to be an effective and safe treatment for early-stage MF, with a significant clinical and histopathological response 4, 5.
- Local Radiotherapy or Total Skin Electron Beam Irradiation: Local radiotherapy or total skin electron beam irradiation has been used with success to control advanced skin disease 3.
- Extracorporeal Photopheresis: Extracorporeal photopheresis may also be used successfully, but it is not generally available 3.
- Interferon-alpha and Chemotherapy: Once the disease becomes refractory to topical therapy, IFN-alpha single-agent or combination chemotherapy may be administered, but the duration of response is often less than 1 year and ultimately all patients will relapse and become refractory 3.
- Retinoids and Immunotherapeutic Agents: There is a great need for the further development of novel emerging treatment modalities, such as retinoids (ie, bexarotene) and immunotherapeutic agents (ie, cytokines, tumor vaccines, and monoclonal antibodies), all of which appear to have significant therapeutic potential in patients with MF 3.
Sequencing Therapies in Mycosis Fungoides
The choice of therapy in mycosis fungoides is based on both patient- and lymphoma-specific factors, such as disease characteristics, comorbidities, symptoms and effect on quality of life, potential associated toxicities of therapy, response and tolerance to prior lines of therapy, and convenience and practicality 6.
- Least Toxic to More Toxic Therapies: Generally, therapies are sequenced from least toxic, targeted, nonimmunosuppressive to more toxic, immunosuppressive and from single agent to multiple agents, as necessary 6.
- Maintenance Regimen: If more toxic, immunosuppressive agents are required to alleviate disease burden or symptoms, they are generally used just long enough to control the disease, then transition to a maintenance regimen with less toxic, less immunosuppressive agents 6.
Multidisciplinary Approach to Diagnosis and Therapy
Mycosis fungoides often shows diagnostic difficulties, due to its absence of specific features and lesional polymorphism, and a patient's treatment requires staging 7.
- Skin-Directed Therapy: Skin-directed therapy includes steroids, nitrogen mustard, bexarotene gel, phototherapy UVB, and photochemiotherapy, i.e., total skin electron radiotherapy 7.
- Systemic Therapies: Systemic therapies include retinoids, bexarotene, interferon, histone deacetylase inhibitors, photopheresis, targeted immunotherapy, and cytotoxic chemotherapy 7.
- Multidisciplinary Team Approach: The complexity of mycosis fungoides associated with long-term chronic evolution and multiple therapy based on disease stage needs a multidisciplinary team approach to be treated 7.