Original papers
New trends in the management of mycosis fungoides and Sezary syndrome

Abstract
Primary cutaneous lymphomas (PCLs) are the most common extranodal non-Hodgkin lymphomas. Given their rich cutaneous symptomatology, frequently ambiguous histology, rarity and heterogeneity, they represent a diagnostic and therapeutic challenge. However, rapid progress of understanding the pathogenesis of these malignancies has been observed recently. Hopefully, this will result in the development of new diagnostic and therapeutic strategies. Here, we review new trends in the diagnostics and therapy of PCL. Particularly rapid progress is observed in mycosis fungoides, the most common T-cell PCL. Based on the newest treatment recommendations for mycosis fungoides, therapy should be started in the early stages. As no curative therapy is currently available, the major goal of treatment is prevention or delay of progression from early disease stages to the later ones. Patients require long-term, sequential therapies with as little toxicity as possible. Skin directed therapy generally produces a good response in the early stages. As the disease progresses, a systemic therapy with immunological modifiers such as bexarotene, interferon a, denileukin diftitox or methotrexate should be applied. Non-responders have to be treated with chemotherapeutic agents, starting with purine analogues and progressing to polychemotherapy. Combined therapy seems to improve response rates, safety and tolerability of treatment. The new therapeutic methods such as histone deacetylase inhibitors, Toll-like receptor agonists, forodesine, third generation adenoviral vector systems, alemtuzumab, zanolimumab are also currently being investigated.
Key words: mycosis fungoides, immunomodulators, primary cutaneous T-cell lymphomas, skin oriented therapy, Sezary syndrome.