Targeted therapies for cutaneous T-cell lymphomas

Cutaneous T-cell lymphomas (CTCLs) comprise a clinicopathologically heterogeneous group of uncommon non-Hodgkin lymphomas that manifest primarily in the skin but also may involve lymph nodes, blood, bone marrow and viscera. CTCLs are generally considered incurable unless allogeneic stem cell transplantation is implemented. Goals of therapy are to control symptoms, maintain cosmesis and improve survival by maximally reducing the tumor burden. Current treatment consists of skin-directed therapy for early stage disease and systemic therapy for advanced stage or refractory early stage disease. Despite the availability of a number of active systemic therapeutic strategies, including biological therapy, cytotoxic chemotherapy and extracorporeal photophoresis, there is an unmet need for targeted therapies, with favorable therapeutic indices, for the treatment of advanced and refractory CTCLs, which often render patients highly susceptible to infection. This review will discuss targeted therapy for the two most extensively studied subtypes of CTCL, mycosis fungoides and Sézary syndrome.