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老年及继发性急性髓系白血病的遗传改变模式和临床结局。

Genetic alteration patterns and clinical outcomes of elderly and secondary acute myeloid leukemia.

机构信息

Shanghai Institute of Hematology, Department of Hematology, Ruijin Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, China.

出版信息

Hematol Oncol. 2019 Oct;37(4):456-463. doi: 10.1002/hon.2656. Epub 2019 Aug 20.

Abstract

To illustrate the clinical and genetic features of elderly and secondary acute myeloid leukemia (AML) patients, we compared 145 elderly AML (e-AML) and 55 secondary AML (s-AML) patients with 451 young de novo AML patients. Both e-AML and s-AML patients showed lower white blood cell (WBC) and bone marrow (BM) blasts at diagnosis. NPM1, DNMT3A, and IDH2 mutations were more common while biallelic CEBPA and IDH1 mutations were less seen in e-AML patients. s-AML patients carried a higher frequency of KMT2A-AF9. In treatment response and survival, e/s-AML conferred a lower complete remission (CR) rate and shorter duration of event-free survival (EFS) and overall survival (OS) compared with young patients. In multivariate analysis, s-AML was an independent risk factor for OS but not EFS in the whole cohort. Importantly, intensive therapy tended to improve the survival of e/s-AML patients without increasing the risk of early death, and hematopoietic stem cell transplantation (HSCT) could rescue the prognosis of s-AML, which should be recommended for the treatment of fit patients.

摘要

为了阐明老年和继发性急性髓系白血病(AML)患者的临床和遗传特征,我们比较了 145 例老年 AML(e-AML)和 55 例继发性 AML(s-AML)患者与 451 例年轻初发 AML 患者。e-AML 和 s-AML 患者在诊断时的白细胞(WBC)和骨髓(BM)原始细胞数较低。NPM1、DNMT3A 和 IDH2 突变更为常见,而 e-AML 患者中双等位基因 CEBPA 和 IDH1 突变较少见。s-AML 患者携带更高频率的 KMT2A-AF9。在治疗反应和生存方面,与年轻患者相比,e/s-AML 患者的完全缓解(CR)率较低,无事件生存(EFS)和总生存(OS)时间较短。在多变量分析中,s-AML 是全队列 OS 的独立危险因素,但不是 EFS 的独立危险因素。重要的是,强化治疗可能改善 e/s-AML 患者的生存,而不会增加早期死亡的风险,造血干细胞移植(HSCT)可以挽救 s-AML 的预后,应推荐用于适合治疗的患者。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2ff1/6899678/01cbc3756389/HON-37-456-g001.jpg

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