骨髓分裂性综合征 骨髓分裂性综合征
Lionel Adès1, Raphael Itzykson1, Pierre Fenaux1
1Service d'hématologie, Hôpital St Louis (Assistance Publique Hôpitaux de Paris) and Paris 7 University, Paris, France.
Lancet (London, England)
|March 25, 2014
概括
骨髓质疏松症候群是干细胞疾病,导致血细胞计数较低,并可能进展为白血病. 治疗因风险而异,包括低风险患者的生长因子和高风险患者的干细胞移植.
科学领域:
- 血液学 血液学 血液学
- 在瘤学瘤学.
- 干细胞生物学 干细胞生物学
背景情况:
- 骨髓发育综合征 (MDS) 是一种克隆性骨髓干细胞疾病.
- 以无效的血液形成为特征,导致细胞衰竭 (低血细胞计数).
- 大约15%的病例发生在化疗或放射治疗后;在老年人中最常见.
研究的目的:
- 为了提供一个全面的概述骨髓质疏松症候群.
- 讨论病理生理学,临床表现,诊断,预后和治疗策略.
主要方法:
- 对有关骨髓质疏松症候群的现有文献的综述.
- 分析病理机制,包括细胞遗传和遗传因素.
- 检查诊断标准和预后指标.
主要成果:
- 病理生理学涉及细胞遗传变化,基因突变和高甲基化.
- 临床症状源于贫血,感染和由于细胞衰竭的出血.
- 诊断依赖于血液/骨髓检查,揭示出发育不良和潜在的爆发过量.
结论:
- 预后是由爆发百分比,细胞衰竭和细胞遗传异常决定的.
- 较低风险的MDS治疗包括生长因子,利那利多米德和输血.
- 较高风险的MDS治疗包括低甲基化剂和全原干细胞移植.
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