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Blood, 12 November 2009, Vol. 114, No. 20, pp. 4337-4353. Prepublished online as a Blood First Edition Paper on August 20, 2009; DOI 10.1182/blood-2009-07-202895. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2009-07-202895v1 114/20/4337    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Prince, H. M. Articles by Hoppe, R. T. PubMed PubMed Citation Articles by Prince, H. M. Articles by Hoppe, R. T. Related Collections How I Treat Free Research Articles Lymphoid Neoplasia Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HOW I TREAT How I treat mycosis fungoides and Sézary syndrome H. Miles Prince1, Sean Whittaker2, and Richard T. Hoppe3 1 Division of Haematology and Medical Oncology, Peter MacCallum Cancer Centre and University of Melbourne, Victoria, Australia; 2 St John's Institute of Dermatology, Guy's and St Thomas' NHS Foundation Trust and Division of Genetics and Molecular Medicine, King's College London, London, United Kingdom; and 3 Department of Radiation Oncology, Stanford University, CA The most common subtypes of primary cutaneous T-cell lymphomas are mycosis fungoides (MF) and Sézary syndrome (SS). The majority of patients have indolent disease; and given the incurable nature of MF/SS, management should focus on improving symptoms and cosmesis while limiting toxicity. Management of MF/SS should use a "stage-based" approach; treatment of early-stage disease (IA-IIA) typically involves skin directed therapies that include topical corticosteroids, phototherapy (psoralen plus ultraviolet A radiation or ultraviolet B radiation), topical chemotherapy, topical or systemic bexarotene, and radiotherapy. Systemic approaches are used for recalcitrant early-stage disease, advanced-stage disease (IIB-IV), and transformed disease and include retinoids, such as bexarotene, interferon-, histone deacetylase inhibitors, the fusion toxin denileukin diftitox, systemic chemotherapy including transplantation, and extracorporeal photopheresis. Examples of drugs under active investigation include new histone deacetylase inhibitors, forodesine, monoclonal antibodies, proteasome inhibitors, and immunomodulatory agents, such as lenalidomide. It is appropriate to consider patients for novel agents within clinical trials if they have failed front-line therapy and before chemotherapy is used. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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