NCCN Guidelines Insights: Primary Cutaneous Lymphomas, Version 2.2020

• DOI: 10.6004/jnccn.2020.0022
• Publication Date: 2020-05-07T14:20:53Z
• Authors: Neha Mehta-Shah; Steven M. Horwitz; Stephen Ansell; Weiyun Z. Ai; Jeffrey Barnes; Stefan K. Barta; Mark W. Clemens; Ahmet Dogan; Kristopher Fisher; Aaron M. Goodman; Gaurav Goyal; Joan Guitart; Ahmad Halwani; Bradley M. Haverkos; Richard T. Hoppe; Eric Jacobsen; Deepa Jagadeesh; Matthew A. Lunning; Amitkumar Mehta; Elise A. Olsen; Barbara Pro; Saurabh A. Rajguru; Satish Shanbhag; Aaron Shaver; Andrei Shustov; Lubomir Sokol; Pallawi Torka; Carlos Torres-Cabala; Ryan Wilcox; Basem M. William; Jasmine Zain; Mary A. Dwyer; Hema Sundar; Youn H. Kim
• Keywords: Romidepsin; Brentuximab vedotin; Systemic therapy; Cutaneous T-cell lymphoma

Mycosis fungoides (MF) is the most common subtype of cutaneous T-cell lymphoma (CTCL), and Sézary syndrome (SS) a rare erythrodermic leukemic CTCL characterized by significant blood involvement. Although early-stage disease can be effectively treated predominantly with skin-directed therapies, systemic therapy often necessary for treatment advanced-stage disease. Systemic options have evolved in recent years approval novel agents such as romidepsin, brentuximab vedotin, mogamulizumab. These NCCN Guidelines Insights discuss diagnosis management MF SS (with focus on therapy).