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!