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1、Myelodysplastic Syndromes Version 1 2019 July 16 2018 NCCN org NCCN Guidelines for Patients available at www nccn org patients NCCN Clinical Practice Guidelines in Oncology NCCN Guidelines Continue Version 1 2019 07 16 18 National Comprehensive Cancer Network Inc 2018 All rights reserved The NCCN Gu
2、idelines and this illustration may not be reproduced in any form without the express written permission of NCCN Peter L Greenberg MD Chair Stanford Cancer Institute Richard M Stone MD Vice Chair Dana Farber Brigham and Women s Cancer Center Aref Al Kali MD Mayo Clinic Cancer Center Rafael Bejar MD P
3、hD UC San Diego Moores Cancer Center John M Bennett MD Consultant Andrew M Brunner MD Massachusetts General Hospital Cancer Center Carlos M De Castro MD Duke Cancer Institute H Joachim Deeg MD Fred Hutchinson Cancer Research Center Seattle Cancer Care Alliance Amy E DeZern MD MHS The Sidney Kimmel C
4、omprehensive Cancer Center at Johns Hopkins Olga Frankfurt MD Robert H Lurie Comprehensive Cancer Center of Northwestern University Karin Gaensler MD UCSF Helen Diller Family Comprehensive Cancer Center Guillermo Garcia Manero MD The University of Texas MD Anderson Cancer Center Elizabeth A Griffith
5、s MD Roswell Park Cancer Institute David Head MD Vanderbilt Ingram Cancer Center Ruth Horsfall PhD MSc Patient Advocate Robert A Johnson MD St Jude Children s Research Hospital The University of Tennessee Health Science Center Mark Juckett MD University of Wisconsin Carbone Cancer Center Sioban Keel
6、 MD Fred Hutchinson Cancer Research Center Seattle Cancer Care Alliance Virginia M Klimek MD Memorial Sloan Kettering Cancer Center Rami Komrokji MD Moffitt Cancer Center Patricia Kropf MD Fox Chase Cancer Center Qing Li MD PhD University of Michigan Comprehensive Cancer Center Lori J Maness MD Fred
7、 positive prognostic factor isolated SF3B1 can refine the prognosis of MDS in patients risk stratified by the IPSS or IPSS R and may be helpful in patients predicted to have intermediate risk Consider molecular testing for JAK2 mutation in MDS patients with thrombocytosis See Genes Frequently Somati
8、cally Mutated in MDS MDS C and Discussion Footnote e is new Constitutional mutations predisposing to hematologic malignancy are found in some patients with cytopenias with or without MDS eg GATA2 deficiency syndrome Shwachman Diamond syndrome telomere biology disorder and others Patients harboring t
9、hese constitutional ie germline mutations can present to both pediatric and adult care centers Fanconi anemia is evaluated by chromosome breakage analysis Serum pancreatic isoamylase pediatric and adult patients and serum trypsinogen pediatric patients are often low in Shwachman Diamond syndrome Tel
10、omere biology disorders such as dyskeratosis congenita demonstrate shortened telomere lengths which can be measured by FISH assays using leukocyte samples Erythrocyte adenosine deaminase is often elevated in Diamond Blackfan anemia See Hereditary myeloid malignancy syndromes See Genes Associated wit
11、h Hereditary Myeloid Malignancies MDS C page 5 of 7 Modified footnote f In younger patients CSA is due to disordered mitochondrial heme synthesis often with distinctive mutational and clinical features Some of these patients will respond to pyridoxine or thiamine CSA is not MDS Fleming MD ASH Educat
12、ion Book vol 2011 1 525 531 Consider congenital bone marrow syndromes eg dyskeratosis congenita Shwachman Diamond syndrome CSA may appear late due to lyonization in X linked sideroblastic anemia not limited to younger patients MDS 2 Additional testing Modified the first bullet Consider flow cytometr
13、y FCM for MDS as a diagnostic aid to assess for possible and consider FCM to evaluate for large granular lymphocyte LGL disease and to evaluate for paroxysmal nocturnal hemoglobinuria PNH clone Removed Consider additional genetic screening for patients with familial cytopenias particularly for young
14、er patients Added a new bullet CMV safe CMV negative or leukpheresed blood products are recommended whenever possible for CMV negative transplant candidates Footnote o is new to the page Mutation panel may be useful in this context to validate indolent myeloid hematopoietic disorders Added the follo
15、wing reference to footnote l Dezern AE and Borowitz MJ ICCS ESCCA consensus guidelines to detect GPI deficient cells in paroxysmal nocturnal hemoglobinuria PNH and related disorders part 1 clinical utility Cytometry B Clin Cytom 2018 Jan 94 1 16 22 Updates in Version 1 2019 of the NCCN Guidelines fo
16、r Myelodysplastic Syndromes from Version 2 2018 include Continued NCCN Guidelines Index Table of Contents Discussion Version 1 2019 07 16 18 National Comprehensive Cancer Network Inc 2018 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 1 2019 Myelodysplastic Syndromes Printed by Maria Chen on 7 16 2018 11 56 01 PM For personal use only Not approved for distribution Copyright 201
