NCCN Guidelines Insights: Primary Cutaneous Lymphomas, Version 2.2020
Neha Mehta–Shah, Sarah McCue Horwitz, Stephen M. Ansell, Weiyun Z. Ai, Jeffrey A. Barnes, Stefan K. Barta, Mark W. Clemens, Ahmet Doǧan, Kristopher Fisher, Aaron M. Goodman, Gaurav Goyal, Joan Guitart, Ahmad Halwani, Bradley M. Haverkos, Richard T. Hoppe, Eric D. Jacobsen, Deepa Jagadeesh, Matthew A. Lunning, Amitkumar Mehta, Elise A. Olsen, Barbara Pro, Saurabh Rajguru, Satish Shanbhag, Aaron C. Shaver, Andrei R. Shustov, Lubomir Sokol, Pallawi Torka, Carlos A. Torres‐Cabala, Ryan A. Wilcox, Basem M. William, Jasmine Zain, Mary A. Dwyer, Hema Sundar, Youn H. Kim
Abstract
Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma (CTCL), and Sézary syndrome (SS) is a rare erythrodermic and leukemic subtype of CTCL characterized by significant blood involvement. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy is often necessary for the treatment of advanced-stage disease. Systemic therapy options have evolved in recent years with the approval of novel agents such as romidepsin, brentuximab vedotin, and mogamulizumab. These NCCN Guidelines Insights discuss the diagnosis and management of MF and SS (with a focus on systemic therapy).