Sézary syndrome: diagnostic criteria and therapeutic options.

Sézary syndrome (SS) is a rare form of erythrodermic cutaneous T-cell lymphoma with hematological involvement and a poor prognosis. Therapies include photopheresis, with or without interferon, chemotherapy, and total skin electron beam therapy. The lack of any randomized studies makes it difficult to assess the effect of current therapy on survival. In addition, the different response rates reported for individual treatments may depend as much on the criteria used to define SS as the therapy itself. This article reviews the diagnostic tests that are needed to reliably diagnose SS and offers a critical analysis of current treatment options.