Cutaneous T cell lymphoma-mycosis fungoides and Sezary syndrome: an update.

Mycosis fungoides (MF) is the most common form of cutaneous T cell lymphoma (CTCL) and, for unclear reasons, its incidence appears to be increasing. Due to significant differences in biological behavior, cutaneous T cell lymphomas have recently been reclassified. MF and Sezary syndrome (SS) are now separately classified as indolent and aggressive CTCLs, respectively. The cause of MF and SS remains elusive. Because establishing the diagnosis of MF, particularly in early stages of disease, is often difficult, a guideline with clinical and histological diagnostic criteria has recently been proposed. With the exception of very early stage disease, no cure is available. However, the number of available therapeutic options has increased considerably in recent years, and partial and complete remissions are achievable. Prognosis and selection of appropriate therapy largely depends on stage of disease. A detailed practice guideline with an algorithmic, stage-based approach to therapy has recently been put forth and will likely aid clinicians in managing patients with MF and SS. Despite the indolent nature in most individuals, the disease has a tremendous psychological impact, not only because of the visible nature of the skin lesions, but also due to the rarity of the disease and its chronicity. Knowledge of this disease, therapeutic options and expectations of therapy will greatly enhance care of afflicted patients.