| Title | How we treat mycosis fungoides and Sezary syndrome. | | Author(s) | Prince HM, Whittaker S, Hoppe RT | | Institution | Division of Haematology and Medical Oncology, Peter MacCallum Cancer Centre and University of Melbourne, Melbourne, Victoria, Australia. | | Source | Blood 2009 Aug 20. | | Abstract | The most common subtypes of primary cutaneous T cell lymphomas are mycosis fungoides (MF) and Sezary 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 utilize a 'stage-based' approach; treatment of early-stage disease (IA-IIA) typically involves skin directed therapies which include topical corticosteroids, phototherapy (PUVA or UVB), 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 alfa, histone deacetylase inhibitors (HDACi), the fusion toxin denileukin diftitox, systemic chemotherapy including transplantation, and extracorporeal photopheresis. Examples of drugs under active investigation include new HDACi, forodesine, monoclonal antibodies, proteasome inhibitors and immunomodulatory agents such as lenalidomide. It is appropriate to consider patients for clinical trials if they have failed front-line therapy and before chemotherapy is used. | | Language | ENG | | Pub Type(s) | JOURNAL ARTICLE
| | PubMed ID | 19696197 |
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