Journal of the American Academy of Dermatology
Continuing medical educationPrimary cutaneous T-cell lymphoma (mycosis fungoides and Sézary syndrome): Part II. Prognosis, management, and future directions
Section snippets
Evaluation of a patient, staging, prognosis
Key points Patient evaluation requires a multidisciplinary team approach with dermatologists, oncologists, dermatopathologists, and radiation oncologists Staging of a patient requires an assessment of skin, lymph node, viscera, and blood involvement The prognosis of mycosis fungoides in most patients with limited patch/plaque disease is favorable and similar to that of an age-, sex-, and race-matched control population
Skin-directed therapies
Key points Topical corticosteroids are the most common treatment used in early mycosis fungoides and serve as an adjunct to other topical and systemic therapies at all stages Topical nitrogen mustard and phototherapy have similar efficacy in early stage mycosis fungoides with maintenance therapy needed for prolonged complete remissions Total skin electron beam therapy at a standard dose (30 Gy) is an effective treatment in refractory/relapsed extensive plaque and tumor mycosis fungoides associated with
Systemic therapies
Key points Single-agent systemic therapy (eg, bexarotene) is often used after skin-directed therapy is inadequate or in cases of advanced disease Immunomodulators, such as interferons and retinoids, are commonly used as first-line monotherapy in advanced mycosis fungoides and are also used in low-dose combination with topical agents Histone deactylase inhibitors (vorinostat or romidepsin) are also effective single agents in skin, nodal, and blood disease Alemtuzumab is particularly active in erythrodermic
Antifolates
The reduced folate carrier type 1, an oncofetoprotein that is predominantly expressed in the membranes of fetal and tumor cells, mediates the cellular uptake of folates and antifolate drugs, including methotrexate and a newer agent, pralatrexate (which is approved by the US Food and Drug Administration for relapsed/refractory peripheral T-cell lymphoma).161, 162 Both antifolates are substrates for folylpolyglutamate synthetase and potently inhibit dihydrofolate reductase.163
Low-dose
General health care
Key points Important quality of life considerations include pruritus, xerosis, and the prevention of skin infections Treatment-related toxicities may require dose adjustments, particularly in the elderly, patients with advanced disease, and patients with multiple comorbidities
Many patients are disabled by their pruritus and skin appearance. Emollients should be used for dryness and scaling, and the application of midpotency steroids, particularly triamcinolone 0.1% ointment once or twice daily, is
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