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Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology

Jason Gotlib, Aaron T. Gerds, Prithviraj Bose, Mariana C. Castells, Michael W. Deininger, Ivana Gojo, Krishna Gundabolu, Gabriela Hobbs, Catriona Jamieson, Brandon McMahon, Sanjay R. Mohan, Vivian Oehler, Stephen Oh, Eric Padron, Philip Pancari, Nikolaos Papadantonakis, Animesh Pardanani, Nikolai Podoltsev, Raajit Rampal, Erik Ranheim, Lindsay Rein, David S. Snyder, Brady L. Stein, Moshe Talpaz, Swapna Thota, Martha Wadleigh, Katherine Walsh, Mary Anne Bergman, and Hema Sundar

Mastocytosis is a group of heterogeneous disorders resulting from the clonal proliferation of abnormal mast cells and their accumulation in the skin and/or in various extracutaneous organs. Systemic mastocytosis is the most common form of mastocytosis diagnosed in adults, characterized by mast cell infiltration of one or more extracutaneous organs (with or without skin involvement). The identification of KIT D816V mutation and the emergence of novel targeted therapies have significantly improved the diagnosis and treatment of systemic mastocytosis. However, certain aspects of clinical care, particularly the diagnosis, assessment, and management of mediator-related symptoms continue to present challenges. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnosis and management of patients with systemic mastocytosis.

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Acute Myeloid Leukemia, Version 3.2023, NCCN Clinical Practice Guidelines in Oncology

Daniel A. Pollyea, Jessica K. Altman, Rita Assi, Dale Bixby, Amir T. Fathi, James M. Foran, Ivana Gojo, Aric C. Hall, Brian A. Jonas, Ashwin Kishtagari, Jeffrey Lancet, Lori Maness, James Mangan, Gabriel Mannis, Guido Marcucci, Alice Mims, Kelsey Moriarty, Moaath Mustafa Ali, Jadee Neff, Reza Nejati, Rebecca Olin, Mary-Elizabeth Percival, Alexander Perl, Amanda Przespolewski, Dinesh Rao, Farhad Ravandi, Rory Shallis, Paul J. Shami, Eytan Stein, Richard M. Stone, Kendra Sweet, Swapna Thota, Geoffrey Uy, Pankit Vachhani, Carly J. Cassara, Deborah A. Freedman-Cass, and Katie Stehman

Acute myeloid leukemia (AML) is a heterogeneous hematologic malignancy characterized by the clonal expansion of myeloid blasts in the peripheral blood, bone marrow, and/or other tissues. It is the most common form of acute leukemia among adults and accounts for the largest number of annual deaths from leukemias in the United States. Like AML, blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a myeloid malignancy. It is a rare malignancy characterized by the aggressive proliferation of precursors of plasmacytoid dendritic cells that frequently involves the bone marrow, skin, central nervous system, and other organs and tissues. This discussion section focuses on the diagnosis and management of BPDCN as outlined in the NCCN Guidelines for AML.

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Myeloid/Lymphoid Neoplasms with Eosinophilia and TK Fusion Genes, Version 3.2021, NCCN Clinical Practice Guidelines in Oncology

Aaron T. Gerds, Jason Gotlib, Prithviraj Bose, Michael W. Deininger, Andrew Dunbar, Amro Elshoury, Tracy I. George, Ivana Gojo, Krishna Gundabolu, Elizabeth Hexner, Gabriela Hobbs, Tania Jain, Catriona Jamieson, Andrew T. Kuykendall, Brandon McMahon, Sanjay R. Mohan, Vivian Oehler, Stephen Oh, Animesh Pardanani, Nikolai Podoltsev, Erik Ranheim, Lindsay Rein, Rachel Salit, David S. Snyder, Brady L. Stein, Moshe Talpaz, Swapna Thota, Pankit Vachhani, Martha Wadleigh, Katherine Walsh, Dawn C. Ward, Mary Anne Bergman, and Hema Sundar

Eosinophilic disorders and related syndromes represent a heterogeneous group of neoplastic and nonneoplastic conditions, characterized by more eosinophils in the peripheral blood, and may involve eosinophil-induced organ damage. In the WHO classification of myeloid and lymphoid neoplasms, eosinophilic disorders characterized by dysregulated tyrosine kinase (TK) fusion genes are recognized as a new category termed, myeloid/lymphoid neoplasms with eosinophilia and rearrangement of PDGFRA, PDGFRB or FGFR1 or with PCM1-JAK2. In addition to these aforementioned TK fusion genes, rearrangements involving FLT3 and ABL1 genes have also been described. These new NCCN Guidelines include recommendations for the diagnosis, staging, and treatment of any one of the myeloid/lymphoid neoplasms with eosinophilia (MLN-Eo) and a TK fusion gene included in the 2017 WHO Classification, as well as MLN-Eo and a FLT3 or ABL1 rearrangement.

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NCCN Guidelines® Insights: Myelodysplastic Syndromes, Version 3.2022

Featured Updates to the NCCN Guidelines

Peter L. Greenberg, Richard M. Stone, Aref Al-Kali, John M. Bennett, Uma Borate, Andrew M. Brunner, Wanxing Chai-Ho, Peter Curtin, Carlos M. de Castro, H. Joachim Deeg, Amy E. DeZern, Shira Dinner, Charles Foucar, Karin Gaensler, Guillermo Garcia-Manero, Elizabeth A. Griffiths, David Head, Brian A. Jonas, Sioban Keel, Yazan Madanat, Lori J. Maness, James Mangan, Shannon McCurdy, Christine McMahon, Bhumika Patel, Vishnu V. Reddy, David A. Sallman, Rory Shallis, Paul J. Shami, Swapna Thota, Asya Nina Varshavsky-Yanovsky, Peter Westervelt, Elizabeth Hollinger, Dorothy A. Shead, and Cindy Hochstetler

The NCCN Guidelines for Myelodysplastic Syndromes (MDS) provide recommendations for the evaluation, diagnosis, and management of patients with MDS based on a review of clinical evidence that has led to important advances in treatment or has yielded new information on biologic factors that may have prognostic significance in MDS. The multidisciplinary panel of MDS experts meets on an annual basis to update the recommendations. These NCCN Guidelines Insights focus on some of the updates for the 2022 version of the NCCN Guidelines, which include treatment recommendations both for lower-risk and higher-risk MDS, emerging therapies, supportive care recommendations, and genetic familial high-risk assessment for hereditary myeloid malignancy predisposition syndromes.