骨髓增生异常综合征［精］.ppt

Myelodysplastic syndromes (MDS)
骨髓增生异常综合征
Chen Yun, MD, PhD
陈昀
Professor of Shandong University
Jinan Central Hospital
E-mail: ******@sdu.
Content
Definition(定义)
History(历史)
Etiology(流行病学)
Classifications(分类)
Pathogenesis(发病机理)
Diagnosis and differential diagnosis(诊断)
Treatment(治疗)
Definition
Myelodysplastic syndrome (MDS) is a clonal disorder characterized by ineffective hematopoiesis, which led to either fatal cytopebias or acute myelogenous leukemias (AML) 克隆性疾病、无效造血、致命性血细胞减少症或急性髓细胞白血病演变
ically related to about half of AML cases, especially in older patients 常见于老年人
Clinical features of MDS, are usually presented by bone marrow failure通常表现为骨髓衰竭
Peripheral blood cytopenias bination with a hypercellular bone marrow exhibiting dysplastic changes are the hallmarks of MDS.
History
1941, Bomford and Rhoads—Refractory anemia (RA)
1953, Block—Progression to leukemia (PL)
1956, Björkman—Refractory anemia with ringed sideroblats (RARS)
1970, Dreyfus—Refractory anemia with excess blats (RAEB)
1974, Miescher– Chronic myelomonocytic leukemia (CMML)
1976, FAB cooperative group-the definition of Myelodysplatic syndrome (MDS)
1982, FAB cooperative group-the diagnosis and classification of MDS
1987, Benner—Morphological, Immunogical, and ic classification of MDS
2000, World anization (WHO)—MDS is categorized to myeloid malignances
Etiology
The incidence curves for populations at risk for AML and MDS are similar in shape, with MDS exceeding AML and with a potential increase for both with advancing age.
Similar to AML, the sex distribution of MDS is approximately equal until age 60, after which a substantial male predominance develops.
MDS-related AML, a subtype of AML, mirrors the incidence of AML, including the progressive male predominance that develops with advancing age beyond 60 years.
Classification-WHO
The classification based on morphologic criteria was revised resulting in WHO classification, which provides more homogenous MDS categories but eliminates the “RA