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2010NCCN指南-骨髓增生异常综合征

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2010NCCN指南-骨髓增生异常综合征 NCCN Clinical Practice Guidelines in Oncology™ Myelodysplastic Syndromes V.2.2010 Continue www.nccn.org Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.20...
2010NCCN指南-骨髓增生异常综合征
NCCN Clinical Practice Guidelines in Oncology™ Myelodysplastic Syndromes V.2.2010 Continue www.nccn.org Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. NCCN Myelodysplastic Panel Members Peter L. Greenberg, MD/Chair Stanford Comprehensive Cancer Center Eyal Attar, MD Dana-Farber/Brigham and Women’s Cancer Center | Massachusetts General Hospital Cancer Center Minoo Battiwalla, MD Roswell Park Cancer Institute Harry P. Erba, MD, PhD University of Michigan Comprehensive Cancer Center ‡ ‡ Þ † † † ‡ ‡ † † † John M. Bennett, MD Consultant Clara D. Bloomfield, MD The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solove Research Institute Carlos M. DeCastro, MD Duke Comprehensive Cancer Center H. Joachim Deeg, MD Fred Hutchinson Cancer Research Center/Seattle Cancer Care Alliance Hussain I. Saba, MD, PhD H. Lee Moffitt Cancer Center & Research Institute Paul J. Shami, MD Huntsman Cancer Institute at the University of Utah Kathy Spiers, MD St. Jude Children’s Research Hospital/University of Tennessee Cancer Institute Richard M. Stone, MD Dana-Farber/Brigham and Women’s Cancer Center | Massachusetts General Hospital Cancer Center Martin S. Tallman, MD Robert H. Lurie Comprehensive Cancer Center of Northwestern University Peter Westervelt, MD PhD Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine ‡ ‡ ‡ ‡ † * Writing Committee Member † Medical Oncology ‡ Hematology/Hematology Oncology Þ Internal Medicine � Pathology ‡ ‡ ‡ J † Þ ames M. Foran, MD University of Alabama at Birmingham Comprehensive Cancer Center Guillermo Garcia-Manero, MD The University of Texas M. D. Anderson Cancer Center Steven D. Gore, MD The Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins David Head, MD Vanderbilt-Ingram Cancer Center Lori J. Maness, MD ‡ UNMC Eppley Cancer Center at The Nebraska Medical Center Michael Millenson, MD Fox Chase Cancer Center Tariq Mughal, MD St. Jude Children’s Research Hospital/University of Tennessee Cancer Institute Margaret R. O’Donnell, MD City of Hope Comprehensive Cancer Center † � Stephen D. Nimer, MD † ‡ Memorial Sloan-Kettering Cancer Center ContinueNCCN Guidelines Panel Disclosures Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Table of Contents NCCN Myelodysplastic Syndromes Panel Members Initial Evaluation (MDS-1) The French-American-British (FAB) (MDS-2) World Health Organization (WHO) Classification for de Novo MDS (MDS-2- 3) 4 Treatment of LOW INT-1 (MDS-5 Treatment of INT-2, HIGH (MDS-6 Evaluation and Treatment of Related Anemia (MDS-7 Supportive Care for MDS (MDS-A Guidelines Index Print the Myelodysplastic Syndromes Guideline Summary of Guidelines Updates International Prognostic Scoring System (IPSS) (MDS- ) ) ) ) ) These guidelines are a statement of consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult these 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 makes no representations nor warranties of any kind whatsoever regarding their content, use, or application and disclaims any responsibility for their application or use in any way. These guidelines are copyrighted by National Comprehensive Cancer Network. All rights reserved. These guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2009. Clinical Trials: Categories of Evidence and Consensus: NCCN All recommendations are Category 2A unless otherwise specified. See The believes that the best management for any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. NCCN To find clinical trials online at NCCN member institutions, click here: nccn.org/clinical_trials/physician.html NCCN Categories of Evidence and Consensus For help using these documents, please click here Discussion References Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Summary of the Guidelines updates UPDATES Summary of major changes in the 2.2010 version of the Myelodysplastic Syndromes Guidelines from the 1.2010 version include : The addition of the updated discussion section. Replaced the 2001 World Health Organization (WHO) Classification of MDS with the 2008 version and updated the reference in footnote “i”. Added footnotes “k, l, m, n, and o” with WHO Classification tables. Added high-intensity therapy candidate with footnote “bb” to better define who should receive intensive chemotherapy. Footnote “bb” has been modified to include patient preference. Based on survival data the category of evidence and consensus for azacytidine for the treatment of IPSS category INT-2, High has been changed to a category 1 recommendation, it was previously a category 2A. Added recommendation for azacytidine/decitabine or clinical trial for patients who relapse following allogeneic hemopoietic stem cell transplant. Footnote “ee” is new to the page “While the response rates are similar for both drugs, survival benefit in Phase lll randomized trials is reported for azacytidine and not for decitabine.” Added an extra column recommending clinical trials or supportive care for those that do not respond or relapse after treatment. Summary of major changes in the 1.2010 version of the Myelodysplastic Syndromes Guidelines from the 2.2009 version include : � � � � � � � MDS-2 -3 MDS-6 Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. MDS-1 INITIAL EVALUATION Cytopenia(s), suspect myelodysplasia Required: � � � � � � � � H&P CBC, platelets, differential, reticulocyte count Examination of peripheral smear Bone marrow aspiration with iron stain + biopsy + cytogenetics Serum erythropoietin (prior to RBC transfusion) RBC folate, and serum B Serum ferritin ± iron, TIBC Documentation of transfusion history 12 Consider observation to document indolent course vs marked progression of severe cytopenia AML (See NCCN AML Treatment Guidelines) a b Confirm diagnosis of MDS according to FAB and WHO criteria for classification with application of IPSS. Percentage of marrow myeloblasts should be reported. Patients with significant cytopenias and karyotypes t(8;21), t(15;17), and/or inv(16) or variants should be considered AML. . cFamily HLA - evaluation to include all full siblings; unrelated evaluation to include high resolution allele level typing for HLAA, B, C, DR, DQ. To aid the evaluation for improved response to immunosuppressive therapy. To assess possible Fanconi anemia or dyskeratosis congenita. d e See Classification Systems (MDS-2 MDS-4) See NCCN AML Guidelines) . ( and CATEGORY Diagnosis of MDS established based on morphological and clinical criteriaa,b Helpful in Some Clinical Situations: � � � � � � � HLA typing if hemopoietic stem cell transplant (HSCT) candidate HLA typing if indicated for platelet support HIV testing if clinically indicated Evaluate CMML patients for 5q31-33 translocations and/or PDGFR gene rearrangements Consider flow cytometry to evaluate for PNH clone or to assess possible large granular lymphocytic (LGL) disease Consider additional genetic screening for patients with familial cytopenias c e �Consider HLA-DR15 typingd � Consider molecular testing for JAK2 mutation in patients with thrombocytosis See Treatment of Low, INT-1 (MDS-5) INT-2, High (MDS-6) and Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. CLASSIFICATION SYSTEMS FOR DE NOVO MDS (page 1 of 3) 2008 WHO Classification of MDSih Subtype Blood Dysplasia in 10 % of one cell line, < 5% blasts � Bone marrow Refractory cytopenia with unilineage dysplasia (RCUD)j Single or bicytopenia Refractory anemia with ring sideroblasts (RARS) Anemia, no blasts � 15 % of erythroid precursors w/ring sideroblasts, erythroid dysplasia only, < 5 % blasts Dysplasia in 10 % of cells in 2 hematopoietic lineages, ± 15 % ring sideroblasts, < 5 % blasts; � �Refractory cytopenia with multilineage dysplasia (RCMD) Cytopenia(s), < 1 x 10 /L monocytes9 Unilineage or multilineage dysplasia, No Auer rods, 5 % to 9 % blasts Refractory anemia with excess blasts-1 (RAEB-1) Cytopenia(s), 2-4 % blasts,< 1 x 10 /L monocytes � 9 Unilineage or multilineage dysplasia Auer rods ±, 10 % to 19 % blasts Refractory anemia with excess blasts-2 (RAEB-2) Cytopenia(s), 5-19 % blasts, < 1 x 10 /L monocytes 9 Unilineage dysplasia or no dysplasia but characteristic MDS cytogenetics, < 5% blasts Myelodysplastic syndrome, unclassified (MDS-U) Cytopenias Unilineage erythroid dysplasia, isolated del 5(q), < 5 % blasts MDS associated with isolated del(5q) Anemia, platelets normal or increased FAB Classification of MDSgf FAB subtype % of Peripheral blasts % of Bone marrow blasts Refractory anemia (RA) Refractory anemia with ringed sideroblasts (RARS) Refractory anemia with excess blasts (RAEB) Refractory anemia with excess blasts in transformation (RAEB-t) Chronic myelomonocytic leukemia (CMML) (> 1,000 monocytes/mcL blood) < 1 < 1 < 5 < 5 < 5 5-20 � 5 21-30 < 5 5-20 f g h FAB = French-American-British. Bennett JM, Catovsky D, Daniel MT, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol. 1982;51:189-199. WHO = World Health Organization. Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. MDS-2 i j Brunning R, Orazi A, Germing U, et al. Myelodysplastic Syndromes, Chapter 5, in Swerdlow S, Campo E, Harris NL, et al. World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissue, 4th edition. IARC Press, 2008, p 88-103. This category encompasses refractory anemia (RA), Refractory Neutropenia (RN) and Refractory thrombocytopenia (RT). Cases of RN and RT were previously classified as MDS Unclassified. Continued on next page Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Myelodysplastic/Myeloprolifierative Neoplasms (MDS/MPN) WHO Classificationk Subtype Blood Marrow Chronic myelomonocytic leukemia-1 (CMML-1) >1x10 /L monocytes, <5% blasts 9 Dysplasia in 1 hematopoietic line, <10% blasts ≥ CMML-2 >1x10 /L monocytes, 5-19% blasts or Auer rods 9 Dysplasia in 1 hematopoietic line, 10- 19% blasts or Auer rods ≥ Atypical chronic myeloid leukemia (CML), Bcr-Abl 1 negative WBC 13x10 /L, neutrophil precursors >10%, <20% blasts 9 Hypercellular, <20% blasts Juvenile myelomonocytic leukemia (JMML) >1x10 /L monocytes, <20% blasts 9 l >1x10 /L monocytes, <20% blasts 9 l MDS/MPN, unclassifiable ('Overlap syndrome') Dysplasia + myeloproliferative features , No prior MDS or MPN m Dysplasia + myeloproliferative features k l m n o Orazi A, Bennet JM, Germing U, et al, Myelodysplastic/Myeloproliferative Neoplasms, Chapter 4, in Swerdlow S, Campo E, Harris NL, et al. (Eds.). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition. IARC Press, 2008, pp 76-86. Ph negative plus 2 features: Hb F, PB immature myeloid cells, WBC >10x109/L, clonal chromosomal abnormality, GM-CSF hypersensitivity in vitro. For example, thrombocytosis, leukocytosis, splenomegaly. Greater than 20% blasts in PB or marrow. Some cases with 20-29% blasts, especially if arising from MDS, may be slowly progressive and may behave more similar to MDS (RAEB-t by FAB classification) than to overt AML. Arber DA, Brunning RD, Orazi A, et al. Acute myeloid leukaemia with myelodysplasia-related changes, In Chapter 6, Acute Myeloid Leukemia and Related Precursor Neoplasms, in Swerdlow S, Campo E, Harris NL, et al. (Eds.). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th edition. IARC Press, 2008, pp 124-126. ≥ Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Acute myeloid leukemia with myelodysplasia-related changes WHO Classification 1. AML post MDS or MDS/MPN 2. AML with an MDS-related cytogenetic abnormality 3. AML with multilineage dysplasia n o MDS-3 Continued on next page CLASSIFICATION SYSTEMS FOR DE NOVO MDS (page 2 of 3) Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. MDS-4 Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. pIPSS = International Prognostic Scoring System. q r s t This research was originally published in Blood. Greenberg P, Cox C, LeBeau M, et al. International scoring system for evaluating prognosis in myelodysplastic syndromes. Blood 1997;89:2079-2088; Erratum. Blood 1998;91:1100. © the American Society of Hematology. Patients with 20-30 % blasts may be considered as MDS or AML. Cytogenetics: Good = normal, -Y alone, del(5q) alone, del(20q) alone; Poor = complex ( 3 abnormalities) or chromosome 7 anomalies; Intermediate = other abnormalities. [This excludes karyotypes t(8;21), inv16, and t(15;17), which are considered to be AML not MDS.] Cytopenias: neutrophil count <1,800/mcL, platelets < 100,000/mcL, Hb < 10g/dL. � IPSSp,q Prognostic variable Marrow blasts (%) Karyotype Cytopenia r s t Survival and AML evolution Risk category (% IPSS pop.) LOW (33) INT-1 (38) INT-2 (22) HIGH (7) Overall score 0 0.5-1.0 1.5-2.0 � 2.5 Median survival (y) in the absence of therapy 5.7 3.5 1.1 0.4 25% AML progression (y) in the absence of therapy 9.4 3.3 1.1 0.2 Score value 0 1.0 1.5 2.0 < 5 5-10 --- 11-20 Good 21-30 Intermediate Poor 2/30/1 CLASSIFICATION SYSTEMS FOR DE NOVO MDS (page 3 of 3) 0.5 Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. MDS-5 LOW, INT-1 Clinically significant cytopenia(s) Symptomatic anemia del(5q) ± other cytogenetic abnormalities Serum Epo 500 mU/ml� Serum Epo > 500 mU/ml Lenalidomide Good probability to respond to ISTx Antithymocyte globulin (ATG), cyclosporin A u v w z xParticularly Low/INT-1 patients 60 y, or those with hypocellular marrows, HLA-DR15 or PNH clone positivity. Patients lack features listed in footnote x. � y INT-1 patients with severe cytopenias would also be considered candidates. Hemopoietic stem cell transplant: Allogeneic-matched sibling including standard and reduced intensity preparative approaches or matched unrelated donor (MUD). See IPSS Classification System (MDS-4). See Supportive Care (MDS-A) See dosing of hemopoietic cytokines ( ) . .MDS-7 Supportive care as an adjunct to treatment v Thrombocytopenia, neutropenia No response Follow appropriate pathway below Epoetin alfa (rHu EPO) ± G-CSF w or Darbepoetin alfa ± G-CSF No response Azacytidine/ decitabine or Clinical trial Consider lenalidomide or No response Clinical trial or Consider allotransplant for selected INT-1 patientsz No response No response Azacytidine/ decitabine or Clinical trial No response IST or Clinical trial x or Consider allotransplant for selected INT-1 patientsz Progressive Disease - See INT-2, HIGH (MDS-6) IPSS CATEGORYu TREATMENT Poor probability to respond to ISTy Clinical trial or Consider allotransplant for selected INT-1 patientsz Azacytidine/ decitabine or Clinical trial Consider lenalidomide or Follow appropriate pathway below Myelodysplastic Syndromes Guidelines Index MDS Table of Contents Discussion, References Practice Guidelines in Oncology – v.2.2010NCCN ® Version 2.2010 08/26/2009 © 2009 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. INT-2, HIGHaa High-intensity therapy candidatev,bb Transplant candidate and Donor available Yes No Not high-intensity therapy candidatev,bb Azacytidine (preferred) (category 1)/decitabine or Clinical trial ee High-intensity chemotherapy or ff Azacytidine/decitabine or Clinical trial Allogeneic hemopoietic stem cell transplant (HSCT)cc,dd IPSS CATEGORYu TREATMENT u bb cc ee ff v dd aaINT-1 patients with severe cytopenias unresp
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