diagnosis and classification of myelodysplastic ring sideroblasts
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Diagnosis and classification of Diagnosis and classification of myelodysplastic ring myelodysplastic ring
sideroblastssideroblasts
2008.11.2008.11.2828
DI DI 정유진정유진
|Hematologica| 2008;93(11)
11 월 28 일 conference - DI 발표
Objective• MDS 의 진단체계 ? (X)• MDS cases 들 ? (X)
• IWGM-MDS : myeloblast, promyeloblast, ring
sideroblast 의 형태학적 정의
골수이형증골수이형증
• 비효율적 조혈 , 혈구감소증
• 백혈병 ( 거의 대부분 AML) 전 단계 ->formerly known as "preleukemia" 30% 가 AML 로 변한다고 알려져 있음
Before IWGM-MDS ?Before IWGM-MDS ?
• FAB : 1982~• IPSS : risk assessment• WHO : FAB 개정
MDS 의 FAB 분류MDS 의 FAB 분류
Syndrome Peripheral Bone marrow
Refractory anemia Blasts < % Blasts < 5%, ringedsideroblasts < 15%
Refractory anemia with ringed sideroblasts (primary acquired sideroblastic anemia)
High MCV; dimorphic red cell picture
Blasts < 5%, ringedsideroblasts > 15%
Refractory anemia with excess of blasts(RAEB)
Blasts < 5% Blasts < 5-10%
Chronic myelomonocytic leukemia(CMML)
Monocytecount>1.0×109/L;granulocytes often increased; blasts < 5%
Blasts < 20%;promonocytes may be increased
RAEB in transformation
Blasts > 5% or Auer rods present
Blasts 20-30% or Auer robs present
Marrow blast : <5%, 0; 5-10%, 0.5; 11-20%, 1.5; 21-30%, 2.0
Karyotype : good [rpgmpsos (normal, -Y, 5q-, 20q-), 0
poor prognosis (abn 7, ≥3 abn), 1.0
intermediate prognosis (others), 0.5
Cytopenia : Hb <10 g/dL, ANC < 1,500/L, platelet < 100,000/L
0-1, 0; 2-3, 0.5
IPSS 에 의한 MDS 환자의 수명 예측
IPSS 에 의한 MDS 환자의 수명 예측
Overall Score Median Survival (yr)
Low(0)
Intermediate
1 (0.5 or 1.0)
2 (1.5 of 2.0)
High (≥2.5)
5.7
3.5
1.2
0.4
Disease Blood findings Bone marrow findings
Refractory anemia (RA) Anemia Erythroid dysplasia only No or rare blasts < 5% blasts < 15% ringed sideroblasts
Refractory anemia with ringed sideroblasts (RARS) Anemia Erythroid dysplasia only No blasts 15% ringed sideroblasts < 5% blasts Refractory cytopenia with multilineage dysplasia (RCMD)
Cytopenias (bicytopenia or pancytopenia) Dysplasia in 10% of cells in 2 or more myeloid cell lines
No or rare blasts < 5% blasts in marrow No Auer rods No Auer rods < 1 × 109/L monocytes < 15% ringed sideroblasts Refractory cytopenia with multilineage dysplasia and ringed
Cytopenias (bicytopenia or pancytopenia) Dysplasia in 10% of cells in 2 or more myeloid cell lines
sideroblasts (RCMD-RS) No or rare blasts 15% ringed sideroblasts No Auer rods < 5% blasts < 1 × 109/L monocytes No Auer rods
Refractory anemia with excess blasts-1 (RAEB-1) Cytopenias Unilineage or multilineage dysplasia < 5% blasts 5% to 9% blasts No Auer rods No Auer rods < 1 × 109/L monocytes
Refractory anemia with excess blasts-2 (RAEB-2) Cytopenias Unilineage or multilineage dysplasia 5% to 19% blasts 10% to 19% blasts Auer rods ± Auer rods ± < 1 × 109/L monocytes
Myelodysplastic syndrome, unclassified (MDS-U) Cytopenias Unilineage dysplasia in granulocytes or megakaryocytes No or rare blasts < 5% blasts No Auer rods No Auer rods
MDS associated with isolated del(5q) AnemiaNormal to increased megakaryocytes with hypolobated nuclei
< 5% blasts < 5% blasts Platelets normal or increased No Auer rods Isolated del(5q)
WHO classification and criteria for the myelodysplastic syndromes
Blast - FABBlast - FAB
1) Type 1, Type 2- Type 1 은 agranules- type 2 는 scanty granules
2) Goasguen- type3 정의 (FAB 에선 promyelocytes 로 분류되던 것들 )
-> 생존률 match higher!
IWGM-MDS(International Working Group on Morphology of
MDS)
1) Definition of myeloblast - granule(2)/agranule(1)
2) Promyelocytes - Normal promyelocyte: Golgi zone
3) 형태학적 구분 : normal promyelocytes, blasts (granular/agranular – 숫자와 관계없이 ), dysplastic promyleocytes
Sideroblast by IWGM-MDSSideroblast by IWGM-MDS
• Type 1 - 5 개 이하 granule in cytoplasm
• Type 2- 5 개 이상 granule, but not perinuclear
• Type 3(= ring sideroblast) – 5 개 이상이면서 perinuclear
: 핵을 둘러싸고 있거나 핵 둘레의 적어도 1/3 이상 포위해야
Blast - GranuleBlast - Granule
Promyelocyte - Golgi zonePromyelocyte - Golgi zone
Ring sideroblast – 5 Ring sideroblast – 5 granule+perinucleargranule+perinuclear
Thank you for your attention!Thank you for your attention!
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