Microsoft Word - 修正版2 第一部提出.doc
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6 myelodysplastic syndromes, MDS MDS MDS MDS anemia of chronic disordersacd aplastic anemia, AA 8 MDS 2005 Mufti idiopathic cytopenias of uncertain significance, ICUS MDS 2006 MDS A ACD MDS MDS B MDS International Working Group on MDS Morphology, IWG-MDS 2005 IWG-MDS IWG-MDS IWG-MDS IWG-MDS A B 10 World Health OrganizationWHO MDS 10% WHO Valent MDS minimal diagnostic criteria ICUS criteria WHO MDS 10% 15% 2
7 A B A MDS 10%A MDS 10%B MDS criteria 8 MDS MDS I I II III A IV A B V Grade of dysplasia VI Division of cytogenetic findings VII Grade of dysplasiadivision of cytogenetic findings Grade of diagnostic accuracymds Definite Probable Possible 3 ICUS WHO VIII I MDS Possible / ICUS ICUS ICUS ICUS MDS French-American-BritishFAB 5 refractory anemia, RA MDS 6 AA paroxysmal nocturnal hemoglobinuria, PNH AA MDS 7 AA MDS AA A B 10 AA MDS AA MDS MDS ( Definite Probable 3
8 Possible ) PNH
9 I MDS Valent MDS minimal criteria prerequisite criteria 4) AE Table 1 A ICUS B. 20WHO acute myeloid leukemia with recurrent cytogenetic abnormalities 500 IWG-MDS agranular blastsfab type 1 blasts granular blasts FAB type 2 blasts Goasguen type 3 blasts 10) refractory anemia with excess blasts, RAEBacute myeloid leukemia, AML 10 MDSRAEB-2 AML t (8;21) WHO acute myeloid leukemia with recurrent cytogenetic abnormalities 20 WHO AML 11q23 abnormalities VI C /L chronic myelomonocytic leukemia, CMML FABWHO IWG-MDS D. MDS MDS ACD 5
10 E. AA MDS 1.5cm Jamshidi 60 30%60 20% 11, 12) MRI AA MDS II A B Table 2 A MDS 4 hypo-segmented mature neutrophilspseudo Pelger-Huet anomaly, Pelger 2 fine thin degranulation of neutrophilsa- or hypogranular neutrophils, Hypo-Gr 80% micromegakaryocytesmmgk 2 ringed sideroblastsrs 1/3 5 IWG-MDS RS B MDS ACD A 10MDS PAS PAS WHO A B 6
11 IIIA ringed sideroblasts hypo-segmented mature neutrophilspseudo Pelger-Huet anomaly, Pelger 100 degranulation of neutrophilsa- or hypogranular neutrophils, Hypo-Gr 100 Hypo-Gr Hypo-Gr micromegakaryocytesmmgk mmgk mmgk 10 ringed sideroblastsrs 100 RS IVA B A B RS B
12 V Grade of dysplasia High Intermediate Low Minimal Table 3 High A 10%Pelger 10% Hypo-Gr 10% 10%mMgk 10% 2. A 15%RS 15% RS 15% High MDS Intermediate AB Low AB 1 10 Minimal: AB VI Division of cytogenetic findings Abnormal Normal Unknown Abnormal MDS MDS Valent MDS minimal criteria MDS-relateddecisivecriteria 4) Haase 13) 5q--7/7q q- complex others t(8;21)(q22;q22) t(15;17)(q22;q12) inv(16)(p13;q22)t(16;16)(p13;q22) WHO AML with recurrent cytogenetic abnormalities WHO t(8;21)(q22;q22) t(15;17)(q22;q12) inv(16)(p13;q22) t(16;16)(p13;q22) 20% AML 11q23 abnormalities 8
13 Normal Unknown VII Grade of dysplasiadivision of cytogenetic findings MDS diagnostic accuracy Definite Probable Possible 3 ICUS Table 4 MDS Definite 3 (1) 519% High Intermediate Low Abnormal Normal Unknown (2) 04 High Intermediate Low Abnormal (3) Grade of dysplasia: High 019% Abnormal Normal Unknown MDS Probable 04 Intermediate Normal Unknown MDS Possible 04% Low Normal Unknown AA MDS ICUS Valent ICUS criteria 4) 9
14 Normal Unknown Minimal ICUS 6 MDS MDS Possible 6 VIIIWHO WHO Possible 6 10
15 AA MDS 2 Jamshidi MRI 1.5cm MRI MRI MRI MRI / 60 30%60 20% % % 60% AA: AB 10 AB 10 AB 5% MDS 11
16 MDS: MDS Definite Probable Possible 10AB MDS Possible 12
17 Table 1. Table 2. A Granulocytic series hypo-segmented mature neutrophils (Pelger) degranulation (a- or hypogranular neutrophils: Hypo-Gr) Megakaryocytic series micromegakaryocytes (mmgk) Erythroid series ringed sideroblasts (RS) B Granulocytic series small size hypersegmentation pseudo Chediak-Higashi granule Megakaryocytic series non-lobulated nuclei multiple, widely-separated nuclei Erythroid series nucleus cytoplasm budding internuclear bridging karyorrhexis multinuclearity megaloblastoid change vacuolization 13
18 Table 3. High High Pelger 10% Hypo-Gr 10% mmgk 10% 2. RS 15% Intermediate 23 A B 10% Low 1 A B 10% Minimal 13 A B = 19% Pelger : hypo-segmented mature neutrophils Hypo-Gr :degranulation (a- or hypogranular neutrophils) mmgk : micromegakaryocytes RS: ringed sideroblasts Table 4. MDS Definite 519 High, INT, Low Any 04 High, INT, Low Abnormal 04 High Any MDS Probable 04 INT Normal or Unknown MDS Possible 04 Low Normal or Unknown ICUS 04 Minimal or None Normal or Unknown INTIntermediate ICUS idiopathic cytopenia of uncertain significance 14
19 1) Hadnagy C, Laszlo GA. Acquired dyserythropoiesis in liver disease. Br J Haematol 1991; 78: ) Karcher DS, Frost AR. The bone marrow in human immunodeficiency virus (HIV)-related disease. Morphology and clinical correlation. Am J Clin Pathol 1991; 95: ) Brunning RD, Head D, Bennet JM, et al. Myelodysplastic syndromes. ed. Jaffe ES, Harris NL, Stein H et al. World Health Organization Classification of Tumours. Pathology and genetics, Tumour of Haematopoietic and lymphoid tissues. IARC Press, Lyon, 2001; ) Valent P, Horny HP, Bennett JM, et al. Definitions and Standards in the Diagnosis and Treatment of The Myelodysplastic Syndromes: Consensus Statements and Report from a Working Conference. Leuk Res 2007; 31: ) Bennett JM, Catovsky D, Daniel MT, et al. Proposals for the classification of the myelodysplastic syndromes. Br J Haematol 1982; 51: ) Matsuda A, Germing U, Jinnai I, et al. Difference in clinical features between Japanese and German patients with refractory anemia in myelodysplastic syndromes. Blood 2005; 106: ) Wang H, Chuhjo T, Yasue S, et al. Clinical significance of a minor population of paroxysmal nocturnal hemoglobinuria-type cells in bone marrow failure syndrome. Blood. 2002; 100: ) , ) , ) Goasguen J, Bennett J, Cox C, et al. Prognostic implication and characterization of the blast cell population in the myelodysplastic syndrome. Leuk Res. 1991; 15; ) Tuzuner N, Cox C, Rowe JM, et al. Bone marrow cellularity in myeloid stem-cell disorders: impact of age correction. Leuk Res 1994; 18: ) Tuzuner N, Cox C, Rowe JM, et al. Hypocellular myelodysplastic syndromes (MDS): new proposal. Br J Haematol 1995; 91: ) Haase D, Germing U, Schanz J, et al. New insights into the prognostic impact of the karyotype in MDS and correlation with subtypes: evidence from a core dataset of 2124 patients. Blood. 2007; 110:
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66 * 骨髄異形成症候群 MDS 骨髄異形成症候群 MDS Ⅰ クニカルクエスチョン一覧 CQ 1 CQ 2 CQ 3 CQ 4 小児の RA および芽球が 10% 未満の RAEB の治療は何か 小児の RAEBT および芽球が 10% 以上の RAEB の治療は何か JMML の治療は何か小児の Down 症候群にみられる MDS の治療は何か Ⅱ 略語一覧 MDS (Myelodysplastic
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Microsoft Word - MDS診療の参照ガイドH26改訂版（2015-2-20提出）.doc
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*Criteria 1) B-marker positive 2) Variability in nuclear size and shape 3) Giant cells and multinucleated cells
*Criteria 1) B-marker positive 2) Variability in nuclear size and shape 3) Giant cells and multinucleated cells Abbreviation: LC=large cleaved nuclei, LNC=large non-cleaved nuclei, IMBL=immunoblastic nuclei,
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Key words: pure red cell anemia, allogeneic bone marrow transplantation.
Complete Recovery of Hemopoiesis Following Bone Marrow Transplantation in a Patient with Unresponsive Congenital Pure Red Cell Anemia Masami SHIMADA, Hideo MUGISHIMA, Mitsuhiko HARA, Masahiro MISAWA, Hideo
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第 75 回日本血液学会学術集会骨髄系腫瘍 :CML/MPN/MDS EL-16 ガイドライン ( 標準治療 ) MDS 診療のガイド 鈴木隆浩 Key words : Myelodysplastic syndrome, International prognosis scoring system (IPSS), IPSS-R, guideline はじめに骨髄異形成症候群 (MDS) は難治性の血球減少症を呈する骨髄不全症候群の一つであり,1
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