Treatment of mycosis fungoides and Sézary syndrome: recent advances and novel therapies

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Early-stage mycosis fungoides (stage IA–IIA) may be treated with skin-directed therapy (topical corticosteroids, topical mechlorethamine, superficial radiotherapy and phototherapy). Advanced or refractory disease may require systemic therapy, such as chemotherapy, extracorporeal photopheresis (ECP) or immunotherapy. Although chemotherapy produces a good response in cutaneous T-cell lymphoma (CTCL), it is frequently short lived. Morbidity is common with chemotherapy and infection or septicemia may be preterminal. There has been a burst of novel therapies for the treatment of advanced and refractory CTCL over the past decade, including retinoid X receptor-selective retinoids, monoclonal antibodies, immunotherapy and histone deacetylase inhibitors. Most of these therapeutic agents are still being developed and are being used in Phase I–III clinical trials. However, bexarotene, a retinoid X receptor-selective retinoid, was approved for the treatment of CTCL in 1999 and has efficacy in both early and advanced disease. It may be used alone or in combination with psoralen plus UVA (PUVA), ECP or interferon. As with other forms of low-grade lymphoma, CTCLs are responsive to treatments but readily relapse and cure is rarely achieved. Stem cell transplants are being investigated currently as a potential curative therapy in advanced/refractory CTCL in patients with a good performance status.

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