NCCN Guidelines Insights: T-Cell Lymphomas, Version 1.2021
Steven M. Horwitz, Stephen M. Ansell, Weiyun Z. Ai, Jeffrey A. Barnes, Stefan K. Barta, Mark W. Clemens, Ahmet Doǧan, Aaron M. Goodman, Gaurav Goyal, Joan Guitart, Ahmad Halwani, Bradley M. Haverkos, Richard T. Hoppe, Eric D. Jacobsen, Deepa Jagadeesh, Allison Jones, Youn H. Kim, Neha Mehta–Shah, Elise A. Olsen, Barbara Pro, Saurabh Rajguru, Sima Rozati, Jonathan Said, 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
Abstract
Hepatosplenic T-cell lymphoma (HSTCL) is a rare subtype of T-cell lymphoma associated with an aggressive clinical course and a worse prognosis. HSTCL develops in the setting of chronic immune suppression or immune dysregulation in up to 20% of cases and is most often characterized by spleen, liver, and bone marrow involvement. Diagnosis and management of HSTCL pose significant challenges given the rarity of the disease along with the absence of lymphadenopathy and poor outcome with conventional chemotherapy regimens. These Guidelines Insights focus on the diagnosis and treatment of HSTCL as outlined in the NCCN Guidelines for T-Cell Lymphomas.