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Myeloproliferative Neoplasms NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Myeloproliferative Neoplasms Version 2.2018 — September 7, 2017 NCCN.org Continue Version 2.2018, 09/07/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Version 2.2018 Panel Members NCCN Guidelines Index Table of Contents Myeloproliferative Neoplasms Discussion *Ruben Mesa, MD/Chair ‡ Gabriela Hobbs, MD ‡ Vishnu Reddy, MD ≠ Mayo Clinic Cancer Center Massachusetts General Hospital University of Alabama at Birmingham Cancer Center Comprehensive Cancer Center *Catriona Jamieson, MD, PhD/Vice-Chair ‡ UC San Diego Moores Cancer Center Brandon McMahon, MD ‡ Lindsay A. M. Rein, MD ‡ University of Colorado Cancer Center Duke Cancer Institute Ravi Bhatia, MD † ‡ University of Alabama at Birmingham Sanjay R. Mohan, MD ‡ Bart Scott, MD, MS † Comprehensive Cancer Center Vanderbilt-Ingram Cancer Center Fred Hutchinson Cancer Research Center/ Seattle Cancer Care Alliance Michael W. Deininger, MD, PhD ‡ Stephen Oh, MD, PhD ‡ Huntsman Cancer Institute Siteman Cancer Center at Barnes- David S. Snyder, MD ‡ ξ at the University of Utah Jewish Hospital and Washington City of Hope Comprehensive Cancer Center University School of Medicine Christopher D. Fletcher, MD Brady L. Stein, MD, MHS ‡ Þ University of Wisconsin Eric Padron, MD † Robert H. Lurie Comprehensive Cancer Carbone Cancer Center Moffitt Cancer Center Center of Northwestern University Aaron T. Gerds, MD, MS ‡ † Nikolaos Papadantonakis, MD, PhD Moshe Talpaz, MD † Case Comprehensive Cancer Center/ University of Alabama at Birmingham University of Michigan University Hospitals Seidman Cancer Comprehensive Cancer Center Comprehensive Cancer Center Center and Cleveland Clinic Taussig Cancer Institute Philip Pancari, MD ‡ † Srdan Verstovsek, MD, PhD † Fox Chase Cancer Center The University of Texas Ivana Gojo, MD ‡ MD Anderson Cancer Center The Sidney Kimmel Comprehensive Nikolai Podoltsev, MD, PhD ‡ Cancer Center at Johns Hopkins Yale Cancer Center/ Martha Wadleigh, MD ‡ † Smilow Cancer Hospital Dana-Farber/Brigham and Women’s Jason Gotlib, MD, MS ‡ Cancer Center Stanford Cancer Institute Raajit Rampal, MD, PhD ‡ Þ † Memorial Sloan Kettering Cancer Center Eunice S. Wang, MD ‡ Krishna Gundabolu, MBBS ‡ Roswell Park Cancer Institute Erik Ranheim, MD, PhD ≠ Fred & Pamela Buffett Cancer Center ‡ Hematology/Hematology oncology University of Wisconsin † Medical oncology Carbone Cancer Center Þ Internal medicine Mary Anne Bergman NCCN Guidelines Panel Disclosures ξ Bone marrow transplantation Kristina M. Gregory, RN, MSN, OCN ≠ Pathology Hema Sundar, PhD Continue * Discussion Writing Committee Member Version 2.2018, 09/07/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Version 2.2018 NCCN Guidelines Index Table of Contents Myeloproliferative Neoplasms Discussion NCCN Myeloproliferative Neoplasms Panel Members Clinical Trials: NCCN believes that the best Summary of the Guidelines Updates management of any patient with cancer is in a clinical trial. Participation in clinical trials is especially Myeloproliferative Neoplasms: encouraged. • Workup (MPN-1) • Diagnosis and Risk Stratification (MPN-2) To find clinical trials online at NCCN Member Institutions, click here: nccn.org/clinical_trials/physician.html. Myelofibrosis: • Treatment for Low-Risk Myelofibrosis (MF-1) NCCN Categories of Evidence and Consensus: All • Treatment for Intermediate-Risk 1 (INT-1) Myelofibrosis (MF-2) recommendations are category 2A unless otherwise • Treatment for Intermediate-Risk 2 (INT-2) or High-Risk Myelofibrosis indicated. (MF-3) See NCCN Categories of Evidence • Management of MF-Associated Anemia (MF-4) and Consensus. • Disease Progression to Advanced-Phase/AML (MF-5) • Risk Stratification for Patients with Myelofibrosis (MF-A) • Supportive Care (MF-B) • 2013 IWG-MRT AND ELN Response Criteria for MF (MF-C) 2016 WHO Diagnostic Criteria for Primary Myelofibrosis (MPN-A) 2016 WHO Diagnostic Criteria for PV and ET (MPN-B) Polycythemia Vera: Assessment of Symptom Burden (MPN-C 1 of 3) • Treatment for Low-Risk Polycythemia Vera (PV-1) Myeloproliferative Neoplasm Symptom Assessment Form • Treatment for High-Risk Polycythemia Vera (PV-2) (MPN-SAF) (MPN-C 2 of 3) • 2013 IWG-MRT AND ELN Response Criteria for PV (PV-A) Myeloproliferative Neoplasm Symptom Assessment Form: Total Symptom Score (MPN-SAF TSS-10 Items) (MPN-C 3 of 3) Essential Thrombocythemia: Prognostic Significance of Mutations in MPN (MPN-D) • Treatment for Very Low-Risk and Low-Risk ET (ET-1) IWG-MRT Diagnostic Criteria for Post ET/Post PV MF (MPN-E) • Treatment for Intermediate-Risk Essential Thrombocythemia (ET-2) Special Considerations for the Use of Ruxolitinib (MPN-F) • Treatment for High-Risk Essential Thrombocythemia (ET-3) Special Considerations in the Treatment of PV and ET (MPN-G) • 2013 IWG-MRT AND ELN Response Criteria for ET (ET-A) Definition of Resistance/Intolerance to Hydroxyurea (MPN-H) The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2017. Version 2.2018, 09/07/17 © National Comprehensive Cancer Network, Inc. 2017, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. NCCN Guidelines Version 2.2018 Updates NCCN Guidelines Index Table of Contents Myeloproliferative Neoplasms Discussion Updates in Version 2.2018 of the NCCN Guidelines for Myeloproliferative Neoplasms from Version 1.2018 include: MS-1 • The discussion section was updated to reflect the changes in the algorithm. Updates in Version 1.2018 of the NCCN Guidelines for Myeloproliferative Neoplasms from Version 2.2017 include: • Polycythemia Vera (PV) and Essential Thrombocythemia (ET) are new MPN-2 algorithms for this version of the guidelines. Footnotes: • Global: MPN-SAF TSS-10 items has been modified to • "k": "Dynamic International Prognostic Scoring System (DIPSS)-Plus MPN-SAF TSS (MPN-10) is preferred for the risk stratification of myelofibrosis; however, IPSS should be used at diagnosis. DIPSS can be used for risk stratification, MPN-1 if karyotyping is not available. See Risk Stratification for Patients with Workup: Myelofibrosis (MF-A).These risk stratification systems have been studied • "Bone marrow cytogenetics (blood, if bone marrow is inaspirable) and validated only in patients with PMF but clinically have been used (karyotype ± FISH)" (Also for MF-4, MF-5) for the risk stratification of patients with Post-PV or Post-ET MF. Novel • "Molecular testing (blood) for JAK2 V617F mutation; if negative, test for prognostic models are being developed for the risk stratification of post-PV CALR and MPL mutations (for patients with ET and MF) and JAK2 Exon 12 and post-ET MF. See Discussion. (Also for MF-1, MF-2, MF-3) mutations (for patients with PV)" • "l": "The revised International Prognostic Score of Thrombosis for ET Footnotes: (IPSET-Thrombosis) is preferred for the risk stratification of ET (Haider • "c": "Prognostic models incorporating other mutations have been M, Gangat N, Lasho T, et al. Am J Hematol 2016;91:390-394. Barbui T, proposed to identify patients who may be at risk of leukemic Vannucchi AM, Buxhofer-Ausch V, et al. Blood Cancer J 2015;5:e369)" transformation. The role of next-generation sequencing (NGS) to identify (Also for ET-1, ET-2, ET-3) high-risk mutations and the use of the Molecular International Prognostic Scoring System (MIPSS) is less well-established. NGS remains a research MF-1 tool in many situations. However, it may be useful to establish clonality in • The pathway off Symptomatic has been modified: "(Interferon alfa- selected circumstances (eg, "Triple Negative" non-mutated JAK2, MPL, and 2b, peginterferon alfa-2a, or peginterferon alfa-2b) or Hydroxyurea, if CALR)." cytoreduction would be symptomatically beneficial" • "e": "Evaluation for allogeneic HCT is recommended for all patients with intermediate-2-risk (INT-2) and high-risk myelofibrosis and for patients MF-2 with intermediate-1-risk (INT-1) myelofibrosis with low platelet counts and Footnote: complex cytogenetics. Identification of “higher-risk” mutations may be • "k": "Additional molecular marker monitoring including next-generation helpful in the decision-making regarding allogeneic HCT for patients with sequencing (NGS) is recommended for higher-risk patients with primary primary myelofibrosis (PMF)." (Also for MF-2, MF-3) PMF." (Also for MF-3) Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that
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