期刊导航

论文摘要

骨髓增生异常综合征的昨天、今天和明天

Myelodysplastic syndromes: yesterday, today and tomorrow

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收稿日期:2020-03-26          年卷(期)页码:2020,43(03):185-189

期刊名称:国际输血及血液学杂志

Journal Name:International Journal of Blood Transfusion and Hematology

关键字:骨髓增生异常综合征,发育异常,克隆性造血,基因突变,靶向治疗

Key words:Myelodysplastic syndromes|Dysplasia|Clonal hematopoiesis|Gene mutation|Target therapy

基金项目:

中文摘要

骨髓增生异常综合征(MDS)是一组异质性髓系克隆性肿瘤,其主要特征为导致患者血细胞减少、造血细胞发育异常和克隆性造血。目前,流式细胞术(FCM)免疫表型分析和二代基因测序(NGS)技术等,已被推荐作为MDS的常规诊断方法。近年相继提出一系列MDS前驱性疾病,如衰老相关性克隆性造血(ARCH)、潜质未定的克隆性造血(CHIP)、意义未明克隆性血细胞减少症(CCUS)和高度致癌潜能克隆性造血(CHOP),从而使越来越多疑似MDS,但是未满足MDS最低诊断标准的患者得以确诊。随着将合并疾病、患者脆弱性和基因突变等整合至现有MDS预后评分系统,以及新型靶向药物应用于临床,而实现对MDS患者的个体化治疗。

英文摘要

Myelodysplastic syndromes (MDS) are a heterogenous group of clonal myeloid neoplasms that are characterized by cytopenia, hematopoietic cells dysplasia and clonal hematopoiesis. Nowadays, flow cytometry (FCM) and next generation sequencing (NGS) have been added as a recommended tool for diagnostic purposes in MDS. Recently, several potential pre-MDS diseases have been proposed, including aging related clonal hematopoiesis (ARCH), clonal cytopenia of unknown significance (CCUS) and clonal hematopoiesis with high oncogenic potential (CHOP). And then, more and more patients are referred in whom a potential pre-phase of MDS are diagnosed, but definitive criteria of MDS are not fulfilled. It is probable that a practical individualized treatment approach for patients with MDS could be developed with the incorporation of comorbidity, patients′ frailty and gene mutations into existing prognostic score systems in the near future.

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