Therapy of Sézary syndrome

Sézary syndrome (SS) is a lymphoma of skin-homing T cells. SS is defined as desquamative erythroderma involving at least 80% of the skin in addition to lymphadenopathy and leukemic involvement. Patients have a poor prognosis with an estimated 5-year survival of 20-27% and estimated median survival of 14-36 months. The goals of therapy are destruction of the malignant lymphocytes and correction of the immune imbalances caused by the malignant Th2 lymphocytes. Immunotherapeutics, such as interferons, retinoids, extracorporeal photopheresis and combination therapy are first-line therapies because of their favorable side-effect profiles. This review will focus on this interesting and expanding group of treatments. Chemotherapeutics, such as doxorubicin and gemcitabine, and monoclonal antibodies such as alemtuzumab, are second-line therapies owing to the risk of iatrogenic immunosuppression and cumulative toxicity. SS requires combination therapy, utilizing multiple skin-directed and systemic agents. The goal of this article is to discuss the treatment of SS, with a focus on immunotherapies