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1、Myeloproliferative Neoplasms Version 3 2019 September 4 2019 NCCN org NCCN Clinical Practice Guidelines in Oncology NCCN Guidelines Version 3 2019 09 04 19 National Comprehensive Cancer Network Inc 2019 All rights reserved The NCCN Guidelines and this illustration may not be reproduced in any form w
2、ithout the express written permission of NCCN Continue Aaron T Gerds MD MS Chair Case Comprehensive Cancer Center University Hospitals Seidman Cancer Center and Cleveland Clinic Taussig Cancer Institute Jason Gotlib MD MS Vice Chair Stanford Cancer Institute Prithviraj Bose MD The University of Texa
3、s MD Anderson Cancer Center Michael W Deininger MD PhD Huntsman Cancer Institute at the University of Utah Ivana Gojo MD The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins Krishna Gundabolu MBBS Fred MPN 10 MPN C 2 of 2 Prognostic Significance of Mutations in MPN MPN D IWG MRT Diagnostic
4、 Criteria for Post PV Post ET MF MPN E Special Considerations for the Use of JAK Inhibitors Ruxolitinib MPN F 2 of 4 and Fedratinib MPN F 4 of 4 Special Considerations in the Treatment of PV and ET MPN G Definition of Resistance Intolerance to Hydroxyurea MPN H Clinical Trials NCCN believes that the
5、 best management for any patient with cancer is in a clinical trial Participation in clinical trials is especially encouraged To find clinical trials online at NCCN Member Institutions click here nccn org clinical trials clinicians aspx NCCN Categories of Evidence and Consensus All recommendations a
6、re category 2A unless otherwise indicated See NCCN Categories of Evidence and Consensus 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 ex
7、pected 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 r
8、esponsibility 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 2019 NCCN Guideline
9、s Index Table of Contents Discussion Version 3 2019 09 04 19 National Comprehensive Cancer Network Inc 2019 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 3 2019 Myeloproliferative
10、 Neoplasms Printed by Maria Chen on 9 15 2019 11 36 40 PM For personal use only Not approved for distribution Copyright 2019 National Comprehensive Cancer Network Inc All Rights Reserved MPN 1 Workup 4th bullet modified FISH or multiplex RT PCR 8th bullet modified Molecular testing blood for JAK2 V6
11、17F mutation if negative test for CALR and MPL mutations for patients with ET and MF and JAK2 exon 12 mutations for patients with PV or molecular testing using multi gene NGS panel that includes JAK2 CALR and MPL 9th bullet modified Assessment of symptom burden using MPN Symptom Assessment form Tota
12、l Symptom Score MPN SAF TSS MPN 10 Footnotes c 1st sentence modified as follows Prognostic models incorporating other mutations have been proposed to identify patients with myelofibrosis MF who may be at risk of leukemic transformation d Assessment of symptoms in provider s office at baseline using
13、MPN Symptom Assessment form MPN SAF is recommended for all patients See Assessment of Symptom Burden MPN C 1 of 2 e See MF 2 and MF 3 Evaluation for allogeneic HCT is recommended for all patients with intermediate 2 risk INT 2 and high risk myelofibrosis and for patients with intermediate 1 risk INT
14、 1 myelofibrosis with low platelet counts and complex cytogenetics Identification of higher risk mutations may be helpful in the decision making regarding allogeneic HCT for patients with primary myelofibrosis PMF See Prognostic Significance of Mutations UPDATES Updates in Version 3 2019 of the NCCN
15、 Guidelines for Myeloproliferative Neoplasms from Version 2 2019 include Continued Updates in Version 1 2019 of the NCCN Guidelines for Myeloproliferative Neoplasms from Version 2 2018 include MS 1 The Discussion section has been updated to reflect the changes in the algorithm Updates in Version 2 2
16、019 of the NCCN Guidelines for Myeloproliferative Neoplasms from Version 1 2019 include MF 1 Footnote e modified See Special Considerations for the Use of Ruxolitinib JAK Inhibitors Also for MF 2 and MF 3 MF 3 Fedratinib was added as a treatment option for intermediate risk 2 INT 2 or high risk MF in patients with platelets 50K Initial treatment category 2B Second line therapy if no response or loss response for patients previously treated with ruxolitinib category 2A Footnote i modified Clinica
