The incidence of acute myeloid leukemia (AML) increases with age, with a median age at diagnosis of 67 years. Older patients have inferior responses to chemotherapy, including not only lower complete remission rates but also short disease-free survival in those who do achieve complete remission. For older patients with a high likelihood of response to chemotherapy, recent data support dose intensification and strong consideration of allogeneic hematopoietic stem cell transplantation. For those unlikely to benefit from chemotherapy because of disease- and/or patient-related factors, novel agents and approaches are being pursued. Agents currently available or under study include the nucleoside analogs clofarabine and sapacitabine, the demethylating agents decitabine and azacitidine, the immunomodulatory agent lenalidomide, and the farnesyl transferase inhibitor tipifarnib. These agents may be administered in the outpatient setting, thus AML in older patients is increasingly becoming an outpatient diagnosis. Additionally, novel agents may prolong survival without inducing complete remissions, and therefore the goals and end points of therapy are also shifting. AML in older patients is a very active current area of investigation.
Maria R. Baer and Ivana Gojo
Ruben Mesa, Catriona Jamieson, Ravi Bhatia, Michael W. Deininger, Aaron T. Gerds, Ivana Gojo, Jason Gotlib, Krishna Gundabolu, Gabriela Hobbs, Rebecca B. Klisovic, Patricia Kropf, Sanjay R. Mohan, Stephen Oh, Eric Padron, Nikolai Podoltsev, Daniel A. Pollyea, Raajit Rampal, Lindsay A. M. Rein, Bart Scott, David S. Snyder, Brady L. Stein, Srdan Verstovsek, Martha Wadleigh, Eunice S. Wang, Mary Anne Bergman, Kristina M. Gregory, and Hema Sundar
Myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET) are a group of heterogeneous disorders of the hematopoietic system collectively known as Philadelphia chromosome–negative myeloproliferative neoplasms (MPNs). The diagnosis and the management of patients with MPNs have evolved since the identification of mutations that activate the JAK pathway (JAK2, CALR, and MPL mutations) and the development of targeted therapies has resulted in significant improvements in disease-related symptoms and quality of life. This manuscript discusses the recommendations outlined in the NCCN Guidelines for the diagnostic workup of MPN (MF, PV, and ET), risk stratification, treatment, and supportive care strategies for the management of MF.
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.
Elizabeth A. Griffiths, Laura M. Alwan, Kimo Bachiashvili, Anna Brown, Rita Cool, Peter Curtin, Mark B. Geyer, Ivana Gojo, Avyakta Kallam, Wajih Z. Kidwai, Dwight D. Kloth, Eric H. Kraut, Gary H. Lyman, Sudipto Mukherjee, Lia E. Perez, Rachel P. Rosovsky, Vivek Roy, Hope S. Rugo, Sumithira Vasu, Martha Wadleigh, Peter Westervelt, and Pamela S. Becker
Hematopoietic growth factors, including erythrocyte stimulating agents (ESAs), granulocyte colony-stimulating factors, and thrombopoietin mimetics, can mitigate anemia, neutropenia, and thrombocytopenia resulting from chemotherapy for the treatment of cancer. In the context of pandemic SARS-CoV-2 infection, patients with cancer have been identified as a group at high risk of morbidity and mortality from this infection. Our subcommittee of the NCCN Hematopoietic Growth Factors Panel convened a voluntary group to review the potential value of expanded use of such growth factors in the current high-risk environment. Although recommendations are available on the NCCN website in the COVID-19 Resources Section (https://www.nccn.org/covid-19/), these suggestions are provided without substantial context or reference. Herein we review the rationale and data underlying the suggested alterations to the use of hematopoietic growth factors for patients with cancer in the COVID-19 era.
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.
Ruben A. Mesa, Catriona Jamieson, Ravi Bhatia, Michael W. Deininger, Christopher D. Fletcher, Aaron T. Gerds, Ivana Gojo, Jason Gotlib, Krishna Gundabolu, Gabriela Hobbs, Brandon McMahon, Sanjay R. Mohan, Stephen Oh, Eric Padron, Nikolaos Papadantonakis, Philip Pancari, Nikolai Podoltsev, Raajit Rampal, Erik Ranheim, Vishnu Reddy, Lindsay A.M. Rein, Bart Scott, David S. Snyder, Brady L. Stein, Moshe Talpaz, Srdan Verstovsek, Martha Wadleigh, Eunice S. Wang, Mary Anne Bergman, Kristina M. Gregory, and Hema Sundar
Myeloproliferative neoplasms (MPNs) are a group of heterogeneous disorders of the hematopoietic system that include myelofibrosis (MF), polycythemia vera (PV), and essential thrombocythemia (ET). PV and ET are characterized by significant thrombohemorrhagic complications and a high risk of transformation to MF and acute myeloid leukemia. The diagnosis and management of PV and ET has evolved since the identification of mutations implicated in their pathogenesis. These NCCN Guideline Insights discuss the recommendations outlined in the NCCN Guidelines for the risk stratification, treatment, and special considerations for the management of PV and ET.
Jeffrey Crawford, Pamela Sue Becker, James O. Armitage, Douglas W. Blayney, Julio Chavez, Peter Curtin, Shira Dinner, Thomas Fynan, Ivana Gojo, Elizabeth A. Griffiths, Shannon Hough, Dwight D. Kloth, David J. Kuter, Gary H. Lyman, Mary Mably, Sudipto Mukherjee, Shiven Patel, Lia E. Perez, Adam Poust, Raajit Rampal, Vivek Roy, Hope S. Rugo, Ayman A. Saad, Lee S. Schwartzberg, Sepideh Shayani, Mahsa Talbott, Saroj Vadhan-Raj, Sumithira Vasu, Martha Wadleigh, Peter Westervelt, Jennifer L. Burns, and Lenora Pluchino
Myeloid growth factors (MGFs) are given as supportive care to patients receiving myelosuppressive chemotherapy to reduce the incidence of neutropenia. This selection from the NCCN Guidelines for MGFs focuses on the evaluation of regimen- and patient-specific risk factors for the development of febrile neutropenia (FN), the prophylactic use of MGFs for the prevention of chemotherapy-induced FN, and assessing the risks and benefits of MGF use in clinical practice.
Featured Updates to the NCCN Guidelines
Pamela Sue Becker, Elizabeth A. Griffiths, Laura M. Alwan, Kimo Bachiashvili, Anna Brown, Rita Cool, Peter Curtin, Shira Dinner, Ivana Gojo, Ashley Hicks, Avyakta Kallam, Wajih Zaheer Kidwai, Dwight D. Kloth, Eric H. Kraut, Daniel Landsburg, Gary H. Lyman, Ryan Miller, Sudipto Mukherjee, Shiven Patel, Lia E. Perez, Adam Poust, Raajit Rampal, Rachel Rosovsky, Vivek Roy, Hope S. Rugo, Sepideh Shayani, Sumithira Vasu, Martha Wadleigh, Kelly Westbrook, Peter Westervelt, Jennifer Burns, Jennifer Keller, and Lenora A. Pluchino
Management of febrile neutropenia (FN) is an integral part of supportive care for patients undergoing cancer treatment. The NCCN Guidelines for Hematopoietic Growth Factors provide suggestions for appropriate evaluation, risk determination, prophylaxis, and management of FN. These NCCN Guidelines are intended to guide clinicians in the appropriate use of growth factors for select patients undergoing treatment of nonmyeloid malignancies. These NCCN Guidelines Insights highlight important updates to the NCCN Guidelines regarding the incorporation of newly FDA-approved granulocyte-colony stimulating factor biosimilars for the prevention and treatment of FN.
Featured Updates to the NCCN Guidelines
Daniel A. Pollyea, Dale Bixby, Alexander Perl, Vijaya Raj Bhatt, Jessica K. Altman, Frederick R. Appelbaum, Marcos de Lima, Amir T. Fathi, James M. Foran, Ivana Gojo, Aric C. Hall, Meagan Jacoby, Jeffrey Lancet, Gabriel Mannis, Guido Marcucci, Michael G. Martin, Alice Mims, Jadee Neff, Reza Nejati, Rebecca Olin, Mary-Elizabeth Percival, Thomas Prebet, Amanda Przespolewski, Dinesh Rao, Farhad Ravandi-Kashani, Paul J. Shami, Richard M. Stone, Stephen A. Strickland, Kendra Sweet, Pankit Vachhani, Matthew Wieduwilt, Kristina M. Gregory, Ndiya Ogba, and Martin S. Tallman
The NCCN Guidelines for Acute Myeloid Leukemia (AML) provide recommendations for the diagnosis and treatment of adults with AML based on clinical trials that have led to significant improvements in treatment, or have yielded new information regarding factors with prognostic importance, and are intended to aid physicians with clinical decision-making. These NCCN Guidelines Insights focus on recent select updates to the NCCN Guidelines, including familial genetic alterations in AML, postinduction or postremission treatment strategies in low-risk acute promyelocytic leukemia or favorable-risk AML, principles surrounding the use of venetoclax-based therapies, and considerations for patients who prefer not to receive blood transfusions during treatment.