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Mycosis Fungoides and the Sézary Syndrome Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Mycosis Fungoides and the Sézary Syndrome

Mycosis fungoides and the Sézary syndrome (MF/SS) are neoplasias of malignant T lymphocytes that usually possess the helper/inducer cell surface phenotype. These kinds of neoplasms initially present as skin involvement and as such have been classified as cutaneous T-cell lymphomas.[1] These types of lymphomas are included in the Revised European-American Lymphoma classification as low-grade T-cell lymphomas, which should be distinguished from other T-cell lymphomas that involve the skin, such as anaplastic large cell lymphoma (CD30 positive), peripheral T-cell lymphoma (CD30 negative, with no epidermal involvement), adult T-cell leukemia/lymphoma (usually with systemic involvement), or subcutaneous panniculitic T-cell lymphoma.[2,3] These histologic types of T-cell lymphomas are discussed in another PDQ summary. (Refer to the PDQ summary on Adult Non-Hodgkin Lymphoma Treatment for more information.) In addition, a number of benign or very indolent conditions can be confused with mycosis fungoides. Consultation with a pathologist who has expertise in distinguishing these conditions is important.[4]

The prognosis of patients with MF/SS is based on the extent of disease at presentation (stage).[5] The presence of lymphadenopathy and involvement of peripheral blood and viscera increase in likelihood with worsening cutaneous involvement and define poor prognostic groups.[6] The median survival following diagnosis varies according to stage. Patients with stage IA disease have a median survival of 20 or more years. The majority of deaths for this group are not caused by, nor are they related to, MF.[7] In contrast, more than 50% of patients with stage III through stage IV disease die of MF, with a median survival of less than 5 years.[5,6,7] A report on 1,798 patients from the National Cancer Institute's Surveillance, Epidemiology, and End Results Program (SEER) database found an increase in second malignancies (standardized incidence ratio of 1.32; 95% confidence interval, 1.15–1.52), especially for Hodgkin and non-Hodgkin lymphoma and for myeloma.[8]

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Typically, the natural history of MF is indolent.[9] Symptoms of the disease may present for long periods, an average of 2 to 10 years, as waxing and waning cutaneous eruptions prior to biopsy confirmation. MF/SS is treatable with available topical and/or systemic therapies. Curative modalities, however, have thus far proven elusive, with the possible exception of patients with minimal disease confined to the skin.

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