骨髓增生异常综合征造血细胞增殖分化异常研究进展  

Abnormal Proliferation and Differentiation of Hematopoietic Cells in Myelodysplastic Syndrome Patients ——Review

作　　者：李丽娟[1] 邵宗鸿[1] 

机构地区：[1]天津医科大学总医院血液科,天津300052

出　　处：《中国实验血液学杂志》2015年第5期1504-1508,共5页Journal of Experimental Hematology

基　　金：天津市自然科学基金(15JCYBJC24300);天津市防癌重大专项(12ZCDZSY17900)

摘　　要：骨髓增生异常综合征(myelodysplastic syndrome,MDS)是一组克隆性造血干/祖细胞疾病,其主要临床特征是病态造血及无效造血、难治性血细胞质/量异常,以及高风险向急性白血病转化。本文通过对这一领域的相关研究进展进行综述,表明MDS存在造血细胞分化异常,与血细胞生成有关的细胞和分子因素与MDS的发病机制相关,这些因素可以影响细胞周期相关基因、转录因子、抗凋亡基因及抑癌基因等,结果导致造血细胞凋亡和增殖过程的失衡、细胞周期失控、DNA修复受损、造血微环境和免疫应答改变等。证实了MDS患者存在骨髓造血细胞增殖和分化异常,患者表现为骨髓一系或多系"无效造血"。Myelodysplastic Syndrome( MDS) is a group of clonal disorders of hematopoietic stem cells characterized by peripheral cytopenia,ineffective hematopoiesis,morphologically apparent multilineage dysplasia,and enhanced risk of evolution towards acute myeloid leukemia( AML). Most of the research findings have verified the abnormal proliferation and differentiation of hematopoietic cells in MDS. The defects of cellular and molecular factors such as transcription factors( GATA-1 ~ GATA-3、FOG1、Pu. 1),growth factors( Epo、G-CSF、GM-CSF) and anti-apoptosis genes ultimately affect the cell cycle regulation and mismatch repair of DNA,changes of hematopoietic microenvironment and immune response. These defects result in ineffective hematopoiesis and dysplasia.

关 键 词：骨髓增生异常综合征 造血细胞 细胞增殖 细胞分化 

分 类 号：R551.3[医药卫生—血液循环系统疾病]