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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
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
Possible ) PNH 8 18 9 4-2 4
I MDS Valent MDS minimal criteria prerequisite criteria 4) AE Table 1 A. 1 13 3 6 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. 110 9 /L chronic myelomonocytic leukemia, CMML FABWHO IWG-MDS D. MDS MDS ACD 5
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
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 25 25 25 mmgk 3 25 10 mmgk 10 ringed sideroblastsrs 100 RS IVA B 100 100 25 A B 25 25 3 25 10 10 100 RS 5 100 B 10 155 10 7
V Grade of dysplasia High Intermediate Low Minimal Table 3 High 1 2 1. A 10%Pelger 10% Hypo-Gr 10% 10%mMgk 10% 2. A 15%RS 15% RS 15% High MDS Intermediate AB 23 10 Low AB 1 10 Minimal: AB 13 19 VI Division of cytogenetic findings Abnormal Normal Unknown Abnormal MDS MDS Valent MDS minimal criteria MDS-relateddecisivecriteria 4) Haase 13) 5q--7/7q- +8 20q- 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
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
6 13 04 Normal Unknown Minimal ICUS 6 MDS MDS Possible 6 VIIIWHO WHO Possible 6 10
AA MDS 2 Jamshidi MRI 1.5cm MRI MRI MRI MRI / 60 30%60 20% 60 3059%60 2059% 60% AA: AB 10 AB 10 AB 5% MDS 11
MDS: MDS Definite Probable Possible 10AB MDS Possible 12
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
Table 3. High High 1 2 1. 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
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