Optimal drug treatment for cutaneous T cell lymphoma

Mycosis fungoides and Sézary syndrome are the most frequent manifestations of cutaneous T cell lymphoma (CTCL). Different treatments are used according to the stage of disease. In the early stages, therapy with psoralens/ultraviolet A (PUVA), alone or combined with retinoids and chlormethine (nitrogen mustard), is most often used. In more advanced stages, electron beam radiation and, more recently, interferon-alpha (IFNalpha) appear to be promising. IFNalpha may be used alone (3 to 9MU 3 times weekly) or combined with retinoids and PUVA therapy. Photophoresis is a recent treatment of particular interest in patients with Sézary syndrome who have not received previous treatment. Finally, chemotherapy has limited indications, more often in patients with advanced stage disease that does not respond to radiotherapy or immunomodulators. The main problem in the treatment of CTCL remains the frequency of relapses, requiring maintenance therapy whose optimum duration and real efficiency has not yet been demonstrated.