From the Guidelines
Mycosis fungoides is a type of cutaneous T-cell lymphoma that requires a comprehensive treatment approach, with the choice of therapy dependent on the extent of cutaneous disease and plaque thickness 1.
Treatment Overview
For early-stage disease (stages IA–IIA), skin-directed therapy is appropriate, including topical therapy, superficial radiotherapy, and phototherapy 1.
- Treatment options for early-stage disease include:
- Topical corticosteroids
- Phototherapy with narrowband UVB or psoralen plus UVA (PUVA)
- For more advanced disease (stage IIB or higher), systemic therapy is required, and options may include chemotherapy regimens, although these generally achieve complete responses in the region of 30% but are short-lived 1.
Advanced Disease Treatment
For advanced disease, immunotherapy and extracorporeal photopheresis (ECP) may be considered, especially for erythrodermic CTCL patients, as responses to chemotherapy are generally poor 1.
- Additional treatment options for advanced disease include:
- Total skin electron beam therapy (TSEBT)
- Localized radiotherapy
- New agents such as bexarotene and denileukin diftitox, which offer important therapeutic alternatives 1. It is essential to monitor patients closely and adjust treatment as needed to manage symptoms and prevent disease progression, with a multidisciplinary approach recommended to ensure optimal care for patients with mycosis fungoides 1.
From the Research
Definition and Characteristics
- Mycosis fungoides (MF) is the most common form of cutaneous T-cell lymphoma, accounting for more than 60% of cases 2.
- It is characterized by skin-homing CD4+ T cells and is notable for highly symptomatic progressive skin lesions, including patches, plaques, tumors, and erythroderma 3.
- MF is a low-grade cutaneous lymphoma with an indolent course, but it can progress to advanced stages with a poorer prognosis 3, 2.
Clinical Presentation
- MF typically presents with cutaneous erythematous patches, plaques, and tumors 4.
- The disease can progress from early-stage patches or plaques to more widespread skin involvement, and in some cases, to advanced stages with skin tumors, extracutaneous spread, and poor prognosis 2.
- Lesions can be located in difficult-to-treat areas, such as the face and intertriginous zones, and can be recalcitrant to recommended therapy 5.
Diagnosis and Staging
- Diagnosis of MF can be challenging due to its nonspecific skin presentation and the absence of specific features 3, 4.
- Skin biopsy for histopathology and immunohistochemical analysis is essential to confirm the diagnosis of MF 6.
- Staging follows the TNMB system, and other factors associated with poorer prognosis include advanced age, male gender, folliculotropism, and elevated lactate dehydrogenase 6.
Treatment
- Treatment of MF is divided into skin-directed therapies (topical treatments, phototherapy, radiotherapy) and systemic therapies (biological response modifiers, targeted therapies, chemotherapy) 2, 6.
- Skin-directed therapies include topical agents, such as mechlorethamine, carmustine, and bexarotene gel, as well as phototherapy and radiotherapy 3, 4.
- Systemic therapies include interferon, retinoids, oral bexarotene, denileukin diftitox, vorinostat, and cytotoxic chemotherapy 3, 4.
- A multidisciplinary team approach is necessary for the management of MF, especially in advanced stages or in cases with complex presentations 4, 5.