1. Diagnosis and classification of myelodysplastic ring sideroblasts
11월 28일 conference - DI 발표
|Hematologica| 2008;93(11)
Diagnosis and classification of myelodysplastic ring sideroblasts
DI 정유진

2. Objective
MDS의 진단체계? (X)
MDS cases들? (X)
IWGM-MDS :
myeloblast, promyeloblast, ring sideroblast의 형태학적 정의

3. 골수이형증 비효율적 조혈, 혈구감소증 백혈병 (거의 대부분 AML) 전 단계
->formerly known as "preleukemia"
30%가 AML로 변한다고 알려져 있음

4. Before IWGM-MDS ?
FAB : 1982~
IPSS : risk assessment
WHO : FAB 개정

5. MDS의 FAB 분류 Syndrome Peripheral Bone marrow Refractory anemia
Blasts < %
Blasts < 5%, ringed
sideroblasts < 15%
Refractory anemia with ringed sideroblasts (primary acquired sideroblastic anemia)
High MCV; dimorphic red cell picture
Blasts < 5%, ringed
sideroblasts > 15%
Refractory anemia with excess of blasts(RAEB)
Blasts < 5%
Blasts < 5-10%
Chronic myelomonocytic leukemia(CMML)
Monocyte count>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

6. IPSS에 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
Marrow blast : <5%, 0; 5-10%, 0.5; 11-20%, 1.5; 21-30%, 2.0
Karyotype : good [rpgmpsos (normal, -Y, 5q-, 20q-), 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; 2-3, 0.5

7. WHO classification and criteria for the myelodysplastic syndromes
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)
No blasts
   15% ringed sideroblasts
Refractory cytopenia with multilineage dysplasia (RCMD)
Cytopenias (bicytopenia or pancytopenia)
Dysplasia in  10% of cells in 2 or more myeloid cell lines
< 5% blasts in marrow
No Auer rods
< 1 × 109/L monocytes
Refractory cytopenia with multilineage dysplasia and ringed
  sideroblasts (RCMD-RS)
  15% ringed sideroblasts
Refractory anemia with excess blasts-1 (RAEB-1)
Cytopenias
Unilineage or multilineage dysplasia
5% to 9% blasts
Refractory anemia with excess blasts-2 (RAEB-2)
5% to 19% blasts
10% to 19% blasts
Auer rods ±
Auer rods ± 
Myelodysplastic syndrome, unclassified (MDS-U)
Unilineage dysplasia in granulocytes or megakaryocytes
MDS associated with isolated del(5q)
Normal to increased megakaryocytes with hypolobated nuclei
Platelets normal or increased
Isolated del(5q)
WHO classification and criteria for the myelodysplastic syndromes

9. Blast - FAB 1) Type 1, Type 2 Type 1은 agranules
type 2는 scanty granules
2) Goasguen- type3 정의 (FAB에선 promyelocytes로 분류되던 것들)
->생존률 match higher!

10. 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

12. Sideroblast by IWGM-MDS
Type 1 - 5개 이하 granule in cytoplasm
Type 2- 5개 이상 granule, but not perinuclear
Type 3(= ring sideroblast) – 5개 이상이면서 perinuclear
: 핵을 둘러싸고 있거나 핵 둘레의 적어도 1/3이상 포위해야

15. Thank you for your attention!
Blast - Granule
Promyelocyte - Golgi zone
Ring sideroblast – 5 granule+perinuclear
Thank you for your attention!