mds诊断与治疗.ppt

MDS的诊断与治疗
陈峰
江苏省血液病研究所
苏州大学附属第一医院血液科
MDS本质是起源于造血干细胞的恶性克隆性疾病。
血细胞减少
无效造血
高风险向AML转化
MDS细胞起源于尚未有系列分化的早期干细of erythroid precursors are ringed sideroblasts < 5% blasts
MDS Classifications(2008)
Disease
Blood findings
Bone marrow findings
Refractory cytopenia with multilineage dysplasia (RCMD)
Cytopenia(s) No or rare blasts (<1%) No Auer rods < 1 x 109/L monocytes
Dysplasia in 10% of cells in two or more myeloid lineages (neutrophil and/or erythroid precursors and/or megakaryocytes) < 5% blasts No Auer rods ± 15% ringed sideroblasts
Refractory anemia with excess blasts-1 (RAEB-1)#
Cytopenia(s) < 5% blasts No Auer rods < 1 x 109/L monocytes
Unilineage or multilineage dysplasia 5–9% blasts No Auer rods
Refractory anemia with excess blasts-2 (RAEB-2)
Cytopenia(s) 5–19% blasts Auer rods ± < 1 x 109/L monocytes
Unilineage or multilineage dysplasia 10–19% blasts Auer rods ±
MDS Classifications(2008)
Disease
Blood findings
Bone marrow findings
Myelodysplastic syndrome – unclassified (MDS-U) §
Cytopenias 1% blasts
Unequivocal dysplasia in less than 10% of cells in one or more myeloid cell lines < 5% blasts
MDS associated with isolated del(5q)
Anemia Usually normal or increased platelet count No or rare blasts (< 1%)
Normal to increased megakaryocytes with hypolobated nuclei < 5% blasts Isolated del(5q) cytogenetic abnormality No Auer rods
MDS Classifications
*Bicytopenia may occasionally be observed. Cases with pancytopenia should be classified as MDS-U
#If the marrow myeloblast percentage is < 5% but there are 2% to 4% myeloblasts in the blood, the diagnostic classification is RAEB-1.
# If the marrow myeloblast percentage is < 5% and there are 1% myeloblasts in the blood, the case should be classified as MDS-U.
§Cases with Auer rods and < 5% myeloblasts in the blood and < 10% in the marrow should be classified as RAEB-2.
MDS/MPN Classifications(2008)
Disease
Blood findings
Bone marrow findings
Chronic myelomo