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).
NCCN Guidelines Insights: Primary Cutaneous Lymphomas, Version 2.2020
Featured Updates to the NCCN Guidelines
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, and Youn H. Kim
NCCN Guidelines Insights: T-Cell Lymphomas, Version 2.2018
Steven M. Horwitz, Stephen M. Ansell, Weiyun Z. Ai, Jeffrey Barnes, Stefan K. Barta, Michael Choi, Mark W. Clemens, Ahmet Dogan, John P. Greer, Ahmad Halwani, Bradley M. Haverkos, Richard T. Hoppe, Eric Jacobsen, Deepa Jagadeesh, Youn H. Kim, Matthew A. Lunning, Amitkumar Mehta, Neha Mehta-Shah, Yahurio Oki, Elise A. Olsen, Barbara Pro, Saurabh A. Rajguru, Satish Shanbhag, Andrei Shustov, Lubomir Sokol, Pallawi Torka, Ryan Wilcox, Basem William, Jasmine Zain, Mary A. Dwyer, and Hema Sundar
Natural killer (NK)/T-cell lymphomas are a rare and distinct subtype of non-Hodgkin's lymphomas. NK/T-cell lymphomas are predominantly extranodal and most of these are nasal type, often localized to the upper aerodigestive tract. Because extranodal NK/T-cell lymphomas (ENKL) are rare malignancies, randomized trials comparing different regimens have not been conducted to date and standard therapy has not yet been established for these patients. These NCCN Guidelines Insights discuss the recommendations for the diagnosis and management of patients with ENKL as outlined in the NCCN Guidelines for T-Cell Lymphomas.
Cancer in People Living With HIV, Version 1.2018, NCCN Clinical Practice Guidelines in Oncology
Erin Reid, Gita Suneja, Richard F. Ambinder, Kevin Ard, Robert Baiocchi, Stefan K. Barta, Evie Carchman, Adam Cohen, Neel Gupta, Kimberly L. Johung, Ann Klopp, Ann S. LaCasce, Chi Lin, Oxana V. Makarova-Rusher, Amitkumar Mehta, Manoj P. Menon, David Morgan, Nitya Nathwani, Ariela Noy, Frank Palella, Lee Ratner, Stacey Rizza, Michelle A. Rudek, Jeff Taylor, Benjamin Tomlinson, Chia-Ching J. Wang, Mary A. Dwyer, and Deborah A. Freedman-Cass
People living with HIV (PLWH) are diagnosed with cancer at an increased rate over the general population and generally have a higher mortality due to delayed diagnoses, advanced cancer stage, comorbidities, immunosuppression, and cancer treatment disparities. Lack of guidelines and provider education has led to substandard cancer care being offered to PLWH. To fill that gap, the NCCN Guidelines for Cancer in PLWH were developed; they provide treatment recommendations for PLWH who develop non–small cell lung cancer, anal cancer, Hodgkin lymphoma, and cervical cancer. In addition, the NCCN Guidelines outline advice regarding HIV management during cancer therapy; drug–drug interactions between antiretroviral treatments and cancer therapies; and workup, radiation therapy, surgical management, and supportive care in PLWH who have cancer.
AIDS-Related Kaposi Sarcoma, Version 2.2019
Erin Reid, Gita Suneja, Richard F. Ambinder, Kevin Ard, Robert Baiocchi, Stefan K. Barta, Evie Carchman, Adam Cohen, Oxana V. Crysler, Neel Gupta, Chelsea Gustafson, Allison Hall, Kimberly L. Johung, Ann Klopp, Ann S. LaCasce, Chi Lin, Amitkumar Mehta, Manoj P. Menon, David Morgan, Nitya Nathwani, Ariela Noy, Lee Ratner, Stacey Rizza, Michelle A. Rudek, Julian Sanchez, Jeff Taylor, Benjamin Tomlinson, Chia-Ching J. Wang, Sai Yendamuri, Mary A. Dwyer, CGC, and Deborah A. Freedman-Cass
As treatment of HIV has improved, people living with HIV (PLWH) have experienced a decreased risk of AIDS and AIDS-defining cancers (non-Hodgkin’s lymphoma, Kaposi sarcoma, and cervical cancer), but the risk of Kaposi sarcoma in PLWH is still elevated about 500-fold compared with the general population in the United States. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for AIDS-Related Kaposi Sarcoma provide diagnosis, treatment, and surveillance recommendations for PLWH who develop limited cutaneous Kaposi sarcoma and for those with advanced cutaneous, oral, visceral, or nodal disease.
NCCN Guidelines Insights: B-Cell Lymphomas, Version 3.2019
Featured Updates to the NCCN Guidelines
Andrew D. Zelenetz, Leo I. Gordon, Jeremy S. Abramson, Ranjana H. Advani, Nancy L. Bartlett, Paolo F. Caimi, Julie E. Chang, Julio C. Chavez, Beth Christian, Luis E. Fayad, Martha J. Glenn, Thomas M. Habermann, Nancy Lee Harris, Francisco Hernandez-Ilizaliturri, Mark S. Kaminski, Christopher R. Kelsey, Nadia Khan, Susan Krivacic, Ann S. LaCasce, Amitkumar Mehta, Auayporn Nademanee, Rachel Rabinovitch, Nishitha Reddy, Erin Reid, Kenneth B. Roberts, Stephen D. Smith, Erin D. Snyder, Lode J. Swinnen, Julie M. Vose, Mary A. Dwyer, and Hema Sundar
Diffuse large B-cell lymphomas (DLBCLs) and follicular lymphoma (FL) are the most common subtypes of B-cell non-Hodgkin’s lymphomas in adults. Histologic transformation of FL to DLBCL (TFL) occurs in approximately 15% of patients and is generally associated with a poor clinical outcome. Phosphatidylinositol 3-kinase (PI3K) inhibitors have shown promising results in the treatment of relapsed/refractory FL. CAR T-cell therapy (axicabtagene ciloleucel and tisagenlecleucel) has emerged as a novel treatment option for relapsed/refractory DLBCL and TFL. These NCCN Guidelines Insights highlight important updates to the NCCN Guidelines for B-Cell Lymphomas regarding the treatment of TFL and relapsed/refractory FL and DLBCL.