Huang Yan, Hu Juan, Lu Ting, Luo Yi, Shi Jimin, Wu Wenjun, Han Xiaoyan, Zheng Weiyan, He Jingsong, Cai Zhen, Wei Guoqing, Huang He, Sun Jie
Bone Marrow Transplantation Center,the First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, Zhejiang 310058, People's Republic of China.
Institute of Hematology, Zhejiang University, Hangzhou, Zhejiang 310058, People's Republic of China.
Cancer Manag Res. 2019 May 8;11:4129-4142. doi: 10.2147/CMAR.S194523. eCollection 2019.
According to the recent National Comprehensive Cancer Network (NCCN) guidelines, the risk level in acute myeloid leukemia (AML) patients with and double mutation (AML ) depends on the allelic ratio of . But despite a low or high allelic ratio of , AML patients belong to the favorable or intermediate risk, for whom allogeneic stem cell transplantation is not obligated. However, some latest studies pointing out that and double mutation patients showed an inferior prognosis, which have raised concern about the risk categorization and more effective treatment of AML patients. A total of 76 patients were selected for coexisting and mutations with normal cytogenetics. The prognostic risk factors were analyzed, and treatment strategies including allogeneic stem cell transplantati1on and chemotherapy were compared. In 76 AML patients, 36.8% of patients had hyperleukocytosis (HL) and mutation was the most common concomitant gene (23.7%). For 53 patients in the complete remission (CR), 22 had received allogeneic hematopoietic stem cell transplantation (allo-HSCT) on first complete remission (CR1). Patients in transplantation group had better overall survival (OS) and disease-free survival (DFS) than chemotherapy only (=0.002 and 0.001, respectively). In multivariable Cox model analyses, HL and mutation were independent adverse prognostic factors (all <0.05) for AML patients. Nevertheless, allo-HSCT was an independent good factor of OS and DFS (=0.001 and 0.000; HR =0.173 and 0.138; 95% CI were 0.062-0.483 and 0.049-0.389). And allo-HSCT could moderately improve the poor prognosis of AML Although, AML patients are categorized as favorable or intermediate risk levels according to recent NCCN and ELN guidelines, these patients should receive allo-HSCT in CR1 for a longer survival. AML patients with mutation had a very poor prognosis, and allo-HSCT could moderately improve their survival.
根据美国国立综合癌症网络(NCCN)近期发布的指南,伴有 和 双突变(AML )的急性髓系白血病(AML)患者的风险水平取决于 的等位基因比例。但是,尽管 的等位基因比例较低或较高,AML 患者仍属于低危或中危,无需进行异基因干细胞移植。然而,一些最新研究指出,伴有 和 双突变的患者预后较差,这引发了对AML 患者风险分类及更有效治疗的关注。共选取了76例伴有 和 突变且细胞遗传学正常的患者。分析了预后危险因素,并比较了包括异基因干细胞移植和化疗在内的治疗策略。在76例AML 患者中,36.8%的患者存在高白细胞血症(HL),且 突变是最常见的伴随基因(23.7%)。对于53例完全缓解(CR)的患者,其中22例在首次完全缓解(CR1)时接受了异基因造血干细胞移植(allo-HSCT)。移植组患者的总生存期(OS)和无病生存期(DFS)均优于单纯化疗组(分别为 =0.002和0.001)。在多变量Cox模型分析中,HL和 突变是AML 患者独立的不良预后因素(均 <0.05)。然而,allo-HSCT是OS和DFS的独立良好因素( =0.001和0.000;HR =0.173和0.138;95%CI分别为0.062 - 0.483和0.049 - 0.389)。并且allo-HSCT可适度改善AML 的不良预后。尽管根据NCCN和欧洲白血病网络(ELN)近期发布的指南,AML 患者被归类为低危或中危,但这些患者应在CR1时接受allo-HSCT以获得更长生存期。伴有 突变的AML 患者预后极差,而allo-HSCT可适度改善其生存期。