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

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

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

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

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

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

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

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!

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-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

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