Abstract
PEARLS Primary cutaneous lymphomas (PCL) are subdivided according to cell lineage. These include cutaneous B-cell lymphomas (CBCL) – 30% and cutaneous T-cell lymphomas (CTCL) – 70%. Overall, 1–1.5 new cases per 100,000/year. Annual age-adjusted incidence of CBCL was 4 per million; CTCL was 6 per million persons (Hoppe et al. 2004; Yahalom et al. 2004). Skin is the most common extranodal site of non-Hodgkin’s lymphoma (NHL), representing 2% of new cases of NHL. Affects older adults (55–60 years), 2:1 male predominance, blacks > whites. Hypothesized links with environmental factors or viral etiology not substantiated (de Jong et al. 2008) Presentation: skin lesions, but long natural history. Median time from skin lesion to diagnosis ∼5 years. Sézary cells: malignant T-cells. Sézary syndrome: erythroderma, lymphadenopathy, and Sézary cells in peripheral blood. Treatment should be tailored to specific subtype of PCL. Unlike other NHL’s, which use the Ann Arbor staging system, Mycosis fungoides/Sézary syndrome (MF/SS) uses a TNM staging system that correlates to prognosis (Kim et al. 2007; Horwitz et al. 2008). EORTC and WHO have classification schemes as well (Willemze et al. 2005; Golling et al. 2008).
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REFERENCES
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Further Readings
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Gillis, A., Bui, T.T., Roach, M. (2010). Cutaneous Lymphomas. In: Hansen, E., Roach, M. (eds) Handbook of Evidence-Based Radiation Oncology. Springer, New York, NY. https://doi.org/10.1007/978-0-387-92988-0_37
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DOI: https://doi.org/10.1007/978-0-387-92988-0_37
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