Diagnosis and classification of myelodysplastic ring sideroblasts

1. 11월 28일 conference - DI 발표 |Hematologica| 2008;93(11) Diagnosis and classification of myelodysplastic ring sideroblasts 2008.11.28 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) 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

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-), 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

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) 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) Anemia Normal to increased megakaryocytes with hypolobated nuclei < 5% blasts < 5% blasts Platelets normal or increased No Auer rods Isolated del(5q)

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