Treatment of Mycosis Fungoides

Introduction to Treatment Approaches

The British Journal of Dermatology recommends a stage-based approach for the treatment of mycosis fungoides, with skin-directed therapies being the cornerstone for early stages (IA-IIA) and systemic therapies reserved for advanced or refractory disease 1, 2

The British Journal of Dermatology suggests that skin-directed therapy is appropriate for patients with early stages of mycosis fungoides (stages IA–IIA) with the choice dependent on extent of cutaneous disease and plaque thickness 1
Topical corticosteroids are a treatment option for early stage mycosis fungoides 2
Topical bexarotene gel and topical carmustine (BCNU) are also treatment options for early stage mycosis fungoides 3
PUVA (psoralen plus UVA) has response rates of 79-88% in stage IA and 52-59% in stage IB 4
Narrow-band UVB is a treatment option for patients with patches or very thin plaques 2
Superficial radiotherapy (2-3 fractions of low energy 80-120 kV) is effective for early localized disease and pagetoid reticulosis 1
Local radiotherapy can be curative in patients with early localized disease and pagetoid reticulosis 2

Patients with stage IIB or higher require some form of systemic therapy, according to the British Journal of Dermatology 1
For patients developing one or a few tumors (stage IIB), additional local radiotherapy suffices, as recommended by the Annals of Oncology 2
TSEB therapy is effective but not sufficient alone for stage IIB disease or those with significant hematological involvement 1
Combination of PUVA and interferon or PUVA and retinoids is a treatment option for patients with extensive plaques and tumors 2
Systemic retinoids, including bexarotene, are a treatment option for intermediate stage disease 1, 5

Erythrodermic CTCL patients should be considered for immunotherapy and extracorporeal photopheresis (ECP) as responses to chemotherapy are generally poor, according to the British Journal of Dermatology 1
ECP has shown overall responses of 35-71% in erythrodermic disease (stage III/IVA) 1
Radiotherapy or TSEB is a treatment option for stage IVA 3
Histone deacetylase inhibitors like vorinostat are a treatment option for advanced disease 2
Fusion toxin denileukin diftitox is a treatment option for advanced disease 2

Chemotherapy is only indicated in patients with effaced lymph nodes or visceral involvement (stage IV) or widespread tumor stage MF that cannot be controlled with skin-targeted and immunomodulating therapies, as recommended by the Annals of Oncology 2
Single-agent options include gemcitabine and liposomal doxorubicin 2
Multi-agent chemotherapy regimens generally achieve complete responses of about 30% but these are short-lived (median duration 3-41 months) 1, 6
Allogeneic stem cell transplantation may be considered in young patients with refractory, progressive MF or SS, according to the Annals of Oncology 2
In treatment-resistant cases of late stage disease, palliative radiotherapy and/or chemotherapy may produce significant short-term benefit, but the patient's quality of life should always be given priority, as recommended by the British Journal of Dermatology 1
Early aggressive chemotherapy is associated with considerable side effects but does not improve survival, according to the Annals of Oncology 2
Autologous stem cell transplantation has shown disappointing results in MF and SS 2
Narrow-band UVB should only be used in patients with patches or very thin plaques, as recommended by the Annals of Oncology 2
Long-term cure may be achieved in localized disease such as pagetoid reticulosis, but patients with multifocal early stage disease are likely to achieve only short-term clinical response with recurrent disease for many years, according to the British Journal of Dermatology 6