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NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines® ) Myelodysplastic Syndromes Version 2.2014 ® NCCN NCCN Guidelines Index MDS Table of Contents Discussion NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines® ) Myelodysplastic Syndromes Version 2.2014 Continue www.nccn.org Version 2.2014, 05/21/13 © National Comprehensive Cancer Network, Inc. 2013, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN® . ® NCCN NCCN Guidelines Version 2.2014 Panel Members NCCN Guidelines Index MDS Table of Contents Myelodysplastic Syndromes Discussion * Peter L. Greenberg, MD/Chair ‡ Karin Gaensler, MD ‡ Margaret R. O’Donnell, MD ‡ Stanford Cancer Institute UCSF Helen Diller Family City of Hope Comprehensive Cancer Center Comprehensive Cancer Center Eyal Attar, MD † ‡ Paul J. Shami, MD ‡ Dana-Farber/Brigham and Women’s Guillermo Garcia-Manero, MD Huntsman Cancer Institute Cancer Center | Massachusetts General The University of Texas at the University of Utah Hospital Cancer Center MD Anderson Cancer Center Brady L. Stein, MD, MHSÞ ‡ John M. Bennett, MD Þ† Steven D. Gore, MD † ‡ Robert H. Lurie Comprehensive Cancer Consultant The Sidney Kimmel Comprehensive Center of Northwestern University Cancer Center at Johns Hopkins Clara D. Bloomfield, MD † Richard M. Stone, MD ‡ The Ohio State University Comprehensive * David Head, MD ¹ Dana-Farber/Brigham and Women’s Cancer Center - James Cancer Hospital Vanderbilt-Ingram Cancer Center Cancer Center | Massachusetts General and Solove Research Institute Hospital Cancer Center Rami Komrokji, MD Uma Borate, MD Moffitt Cancer Center James E. Thompson, MD † ‡ University of Alabama at Birmingham Roswell Park Cancer Institute Comprehensive Cancer Center Lori J. Maness, MD ‡ UNMC Eppley Cancer Center at Peter Westervelt, MD, PhD † Carlos M. De Castro, MD † The Nebraska Medical Center Siteman Cancer Center at Barnes- Duke Cancer Institute Jewish Hospital and Washington Michael Millenson, MD Þ ‡ University School of Medicine H. Joachim Deeg, MD † ‡ Fox Chase Cancer Center Fred Hutchinson Cancer Research * Benton Wheeler, MD Center/Seattle Cancer Care Alliance St. Jude Children’s Research Hospital/ University of Tennessee Cancer Institute Olga Frankfurt, MD ‡ Robert H. Lurie Comprehensive Cancer Center of Northwestern University Specialties Index NCCN Maoko Naganuma, MSc * Writing Committee Member Dorothy A. Shead, MS Continue † Medical Oncology ‡ Hematology/Hematology Oncology Þ Internal Medicine NCCN Guidelines Panel Disclosures ¹ Pathology Version 2.2014, 05/21/13 © National Comprehensive Cancer Network, Inc. 2013, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN® . ® NCCN NCCN Guidelines Version 2.2014 Table of Contents NCCN Guidelines Index MDS Table of Contents Myelodysplastic Syndromes Discussion NCCN Myelodysplastic Syndromes Panel Members Clinical Trials: NCCN believes that the best management for any cancer Summary of Guidelines Updates patient is in a clinical trial. Participation in clinical trials is Initial Evaluation (MDS-1) especially encouraged. Additional Testing and Classification (MDS-2) To find clinical trials online at NCCN Member Institutions, click here: 2008 World Health Organization(WHO) Classification of MDS (MDS-3) nccn.org/clinical_trials/physician.html. Myelodysplastic/Myeloproliferative Neoplasms (MDS/MPN) WHO Classification NCCN Categories of Evidence and (MDS-4) Consensus: All recommendations are category 2A unless otherwise International Prognostic Scoring System and Revised International Prognostic specified. Scoring System (MDS-5) See NCCN Categories of Evidence and Consensus. WHO-Based Prognostic Scoring System for MDS (MDS-6) Prognostic Category Low, Intermediate-1 Treatment (MDS-7) Prognostic Category Intermediate-2, High Treatment (MDS-9) Evaluation of Related Anemia/Treatment of Symptomatic Anemia (MDS-10) Recommendations for Flow Cytometry (MDS-A) Supportive Care (MDS-B) 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. ©2013. Version 2.2014, 05/21/13 © National Comprehensive Cancer Network, Inc. 2013, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN® . ® NCCN NCCN Guidelines Version 2.2014 Updates NCCN Guidelines Index MDS Table of Contents Myelodysplastic Syndromes Discussion Updates in Version 2.2014 of the NCCN Guidelines from Version 1.2014 include: DISCUSSION · The discussion section has been updated to reflect the changes in the algorithm. Updates in Version 1.2014 of the NCCN Guidelines from Version 2.2013 include: MDS-1 MDS-4 · Modified footnote “b”: Confirm diagnosis of MDS according to FAB · Modified footnote “p”: “Examples include thrombocytosis, or WHO criteria for classification with application of IPSS. See leukocytosis, and splenomegaly. In addition, RARS-T has been Classification Systems (MDS-3 and MDS-5). The percentage of suggested as a provisionalMDS/MPN entity for individuals with marrow myeloblasts based on morphologic assessment (aspirate RARS and platelet counts³ 450,000 x109 /L.” [Chapter 4, in Swerdlow smears preferred) should be reported. Flow cytometric estimation of S, Campo E, Harris NL, et al (Eds.). World Health Organization blast percentage should not be used as a substitute for morphology Classification of Tumours of Haematopoietic and Lymphoid Tissues, in this context. In expert hands, expanded flow cytometry may be a 4th edition. IARC Press, 2008, pp 85-86.] useful adjunct for diagnosis in difficult cases (See Discussion section, MS-5-6). MDS-5 · Included Revised International Prognostic Scoring System (IPSS-R) MDS-3 · Added the following footnotes: · Removed French-American-British (FAB) Classification of MDS > Footnote “x”: Greenberg PL, Tuechler H, Schanz J, et al. Revised tables. International Prognostic Scoring System (IPSS-R) for · Added “Refractory anemia with excess blasts in transformation myelodysplastic syndromes. Blood 2012;120:2454-2465. (RAEB-T) to the table with footnote “l”. > Footnote “y”: Cytogenetic risks: Very good = -Y, del(11q); Good = · Footnote l: “In the 2008 WHO classification, RAEB-T (as previously Normal, del(5q), del(12p), del(20q), double including del(5q); classified by the FAB group, Bennett JM, Catovsky D, Daniel MT, et Intermediate = del(7q), +8, +19, i(17q), any other single or double al. Proposals for the classification of the myelodysplastic independent clones; Poor = -7, inv(3)/t(3q)/del(3q), syndromes. Br J Haematol 1982;51:189-199) is classified as AML double including -7/del(7q), complex: 3 abnormalities; Very poor = with myelodysplasia-related changes and may be more akin to MDS Complex: >3 abnormalities. than AML. Refer to Arber DA, Brunning RD, Orazi A, et al. Acute > Included the following websites for accessing the IPSS-R calculator myeloid leukaemia with myelodysplasia-related changes. In Chapter tool: http://www.ipss-r.com or 6. Acute Myeloid Leukemia and Related Precursor Neoplasms, in http://mdsfoundation.org/calculator/index.php. A mobile App for the Swerdlow S, Campo E, Harris NL, et al (Eds.). World Health calculator tool is also available for smartphones at MDS IPSS-R. Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition. IARC, Lyon, 2008, pp 124-126.” Note: All recommendations are category 2A unless otherwise indicated. Continued on next page Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Version 2.2014, 05/21/13 © National Comprehensive Cancer Network, Inc. 2013, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN® . UPDATES ® NCCN NCCN Guidelines Version 2.2014 Updates NCCN Guidelines Index MDS Table of Contents Myelodysplastic Syndromes Discussion Updates in Version 1.2014 of the NCCN Guidelines from Version 2.2013 include: MDS-7 MDS-9 · Added “IPSS-R: Very Low, Low, Intermediate” to the heading. · Added “IPSS-R: Intermediate, High, Very High” to the heading. · Following “Clinically significant cytopenia(s)” added “ or increased · Modifed footnote ll: “Based on age, performance status, major marrow blasts.” comorbid conditions, psychosocial status, patient preference, and · Following “Clinically relevant thrombocytopenia or neutropenia” availability of caregiver. Patients may be taken immediately to added “or increased marrow blasts.” transplant or bridging therapy should be used to decrease marrow · Changed “No response” to “Disease progression/no response.” blasts to an acceptable level prior to transplant.” · Added footnote “bb”: “IPSS-R Intermediate patients may be managed as very low/low
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