Mycosis fungoides and the Sézary syndrome

Mycosis fungoides and the Sézary syndrome represent a heterogeneous group of good-to-intermediate-risk non-Hodgkin lymphomas that have recently been identified as distinct histopathologic and clinical entities by the World Health Organization and European Organization for Research on the Treatment of Cancer lymphoma classification systems. Significant progress has been made in identifying and categorizing patients based on clinical prognostic factors, but there is little information regarding the etiology, molecular biology, and molecular genetics of these diseases. This review outlines recent advances in clinical diagnosis and prognosis as well as novel therapeutic approaches.

A number of reports have further defined clinical prognostic subgroups among early-stage patients and those with circulating Sézary cells. The recent availability and demonstrated efficacy of the oral RXR retinoid, bexarotene, has altered the treatment paradigm of early-stage patients who would not otherwise be exposed to systemic therapies. Novel targeted agents and receptor-directed therapies, including the fusion toxin, denileukin diftitox, histone deacetylase inhibitors, and novel nucleoside analog therapies, have demonstrated promising activity and are undergoing further clinical evaluation. The evolution of immunotherapy has been augmented by studies demonstrating the efficacy of peptide-loaded dendritic cells as well as the use of photopheresis to generate an anti-idiotype cytotoxic T-cell response.

This review will enumerate the most recent findings with respect to clinical staging, prognosis, and treatment of patients with mycosis fungoides and the Sézary syndrome. Novel treatment options will be reviewed and treatment paradigms will be outlined.