What are the treatment options for Mycosis fungoides?

From the Guidelines

Treatment options for mycosis fungoides, a type of cutaneous T-cell lymphoma, depend on the disease stage and extent of skin involvement, with skin-directed therapies being the mainstay for early-stage disease (IA-IIA) and systemic therapies added for more advanced disease (IIB-IV). For early-stage disease, skin-directed therapies include topical corticosteroids (such as clobetasol 0.05% applied twice daily), topical retinoids (bexarotene gel 1% applied once daily), topical chemotherapy (nitrogen mustard 0.01-0.02% ointment or solution daily), phototherapy (narrowband UVB for patch disease or PUVA for thicker plaques, typically 2-3 times weekly for several months), and localized radiation therapy for resistant lesions 1.

Key Considerations

• The choice of therapy depends on the extent of cutaneous disease and plaque thickness, with topical mechlorethamine being effective for patients with limited patches and plaques (stage IA-IB) 1.
• Total skin electron beam therapy (TSEB) can be considered as second-line treatment for stage IB MF that has relapsed or is refractory to other skin-directed therapies, with an overall response rate of 87% and a complete response rate of 18% 1.
• For more advanced disease, systemic therapies such as oral retinoids, interferon alfa, histone deacetylase inhibitors, and monoclonal antibodies may be added, with traditional chemotherapy typically reserved for refractory or rapidly progressive disease 1.
• Allogeneic stem cell transplantation may be considered for young, fit patients with advanced disease, with a reduced-intensity conditioning regimen being recommended 1.

Treatment Goals

• The goal of treatment is to control symptoms, improve quality of life, and achieve remission when possible, as mycosis fungoides is typically chronic but slow-growing.
• Treatment is often chronic and requires regular monitoring for disease progression and side effects, with therapy adjustments based on clinical response.
• Palliative care should be considered for all patients with resistant late-stage disease and those with poor performance status (ECOG > 2) 1.

From the FDA Drug Label

VALCHLOR is indicated for the topical treatment of Stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy. The efficacy of VALCHLOR was assessed in a randomized, multicenter, observer-blind, active-controlled, non-inferiority clinical trial of 260 patients with Stage IA, IB, and IIA mycosis fungoides-type cutaneous T-cell lymphoma (MF-CTCL) who had received at least one prior skin-directed therapy.

The treatment options for Mycosis fungoides include:

• Topical mechlorethamine (VALCHLOR) for Stage IA and IB mycosis fungoides-type cutaneous T-cell lymphoma in patients who have received prior skin-directed therapy 2, 2. Note that the treatment should be guided by the disease stage and the patient's prior therapies.

From the Research

Treatment Options for Mycosis Fungoides

The treatment options for mycosis fungoides (MF) vary depending on the stage and extent of the disease. Some of the treatment options include:

• Topical corticosteroids, such as clobetasol propionate cream 0.05%, which have been shown to be effective in treating early-stage MF with minimal side effects 3
• Phototherapy, including narrow-band UVB (NB-UVB) and psoralen with ultraviolet A (PUVA), which have been used to treat all stages of MF and have a favorable adverse effect profile 4
• Photochemotherapy, such as PUVA, which is a safe and effective first-line therapy for early-stage MF 4
• Systemic chemotherapy, which can be used to treat advanced MF, but often results in recurrences 5
• Monoclonal antibodies, such as alemtuzumab, brentuximab vedotin, and mogamulizumab, which have shown promising results in treating MF 5
• Combination therapies, such as PUVA with interferon-alpha or retinoids, which may be beneficial in certain clinical situations 6
• The 308-nm excimer laser, which has been shown to be a safe and effective therapy for early-stage MF 7

Stages of Mycosis Fungoides and Treatment

The treatment of MF is planned mainly according to the stage and extent of the disease. The stages of MF include:

• Patch stage: treated with skin-directed therapies such as topical steroids, phototherapy, and photochemotherapy 4, 5
• Plaque stage: treated with phototherapy, photochemotherapy, and systemic chemotherapy 4, 5
• Tumor stage: treated with systemic chemotherapy, monoclonal antibodies, and other experimental therapies 5

Combination Therapies for Mycosis Fungoides

Combination therapies have been used to increase the therapeutic efficacy of MF treatment. Some combination therapies that have been studied include:

• PUVA with interferon-alpha or retinoids, which may be beneficial in certain clinical situations 6
• Methotrexate with interferon-alpha or bexarotene, which may increase the overall response rate 6
• Bexarotene with vorinostat or gemcitabine, which did not increase the overall response rate but resulted in more pronounced side effects 6