Runt 相关转录因子 1 基因突变的急性髓性白血病患者的临床特征和预后:单中心回顾性分析

IF 3.3 4区 医学 Q2 HEMATOLOGY
Lin-Ya Wang, Yao Li, Qian Jiang, Hao Jiang, Yu Wang, Lan-Ping Xu, Xiao-Hui Zhang, Kai-Yan Liu, Fei-Fei Tang
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引用次数: 0

摘要

本研究旨在通过比较有或没有RUNX1突变的急性髓性白血病(AML)患者的特征,研究Runt相关转录因子1(RUNX1)突变急性髓性白血病(AML)患者的临床特征和预后。我们采用病例配对法(1:4)对180例急性髓性白血病患者进行了回顾性分析,其中包括36例RUNX1突变型(AML-RUNX1mut)急性髓性白血病患者和144例RUNX1野生型(AML-RUNX1wt)急性髓性白血病患者。与AML-RUNX1wt相比,AML-RUNX1mut的ASXL1频率更高(p mut和AML-RUNX1wt分别为73.1%对68.0%(p = 0.64)和80.7%对71.6%(p = 0.37))。与未接受异基因造血细胞移植(allo-HSCT)的患者相比,接受异基因造血细胞移植(allo-HSCT)的AML-RUNX1mut患者生存率更高(3年OS,84.3%对52.7%;p = 0.006)。多变量分析显示,EZH2突变(p = 0.003)、白细胞(WBC)≥30 × 109 /L(p = 0.036)和年龄≥60岁(p = 0.038)是AML-RUNX1mut患者OS较差的显著独立危险因素;WBC≥30 × 109 /L(p = 0.013)和DNMT3A突变(p = 0.045)是AML-RUNX1mut患者DFS较短的显著独立危险因素。总之,AML-RUNX1mut表现出独特的临床特征,但AML-RUNX1mut与AML-RUNX1wt的生存率相当。EZH2共突变、DNMT3A共突变、高龄和高白细胞计数与AML-RUNX1mut的低生存率有关。Allo-HSCT 可以明显改善 AML-RUNX1mut 的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Clinical characteristics and prognosis of acute myeloid leukemia patients with Runt-related transcription factor 1 mutation: A single-center retrospective analysis

This study aimed to investigate the clinical characteristics and prognosis of Runt-related transcription factor 1 (RUNX1) mutant acute myeloid leukemia (AML) patients by comparing the features of AML patients with or without RUNX1 mutation. We retrospectively analyzed 180 AML patients including 36 AML patients with mutant RUNX1(AML-RUNX1mut) and 144 AML patients with wild-type RUNX1(AML-RUNX1wt) were selected using the case-pair method(1:4). Compared to AML-RUNX1wt, AML-RUNX1mut showed higher frequency of ASXL1 (p < 0.001), SRSF2 (p < 0.001), BCORL1 (p < 0.001), RAS (p = 0.010) mutations, and absent NPM1 mutations (p = 0.022). The 3-year overall survival (OS) and disease-free survival (DFS) of AML-RUNX1mut and AML-RUNX1wt were 73.1% versus 68.0% (p = 0.64) and 80.7% versus 71.6% (p = 0.37), respectively. AML-RUNX1mut receiving allogeneic hematopoietic cell transplantation (allo-HSCT) showed better survival than those who did not receive allo-HSCT (3-year OS, 84.3% vs. 52.7%; p = 0.006). Multivariate analysis showed that EZH2 mutation (p = 0.003), white blood cell (WBC) ≥30 × 109/L (p = 0.036) and age ≥60 years (p = 0.038) were significant independent risk factors for inferior OS of AML-RUNX1mut; WBC ≥30 × 109/L (p = 0.013) and DNMT3A mutation (p = 0.045) were significant independent risk factors for shorter DFS of AML-RUNX1mut. In conclusion, AML-RUNX1mut showed unique clinical characteristics, but the survival between AML-RUNX1mut and AML-RUNX1wt were comparable. EZH2 co-mutation, DNMT3A co-mutation, old age and high WBC count were associated with inferior survival of AML-RUNX1mut. Allo-HSCT can significantly improve the prognosis of AML-RUNX1mut.

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来源期刊
Hematological Oncology
Hematological Oncology 医学-血液学
CiteScore
4.20
自引率
6.10%
发文量
147
审稿时长
>12 weeks
期刊介绍: Hematological Oncology considers for publication articles dealing with experimental and clinical aspects of neoplastic diseases of the hemopoietic and lymphoid systems and relevant related matters. Translational studies applying basic science to clinical issues are particularly welcomed. Manuscripts dealing with the following areas are encouraged: -Clinical practice and management of hematological neoplasia, including: acute and chronic leukemias, malignant lymphomas, myeloproliferative disorders -Diagnostic investigations, including imaging and laboratory assays -Epidemiology, pathology and pathobiology of hematological neoplasia of hematological diseases -Therapeutic issues including Phase 1, 2 or 3 trials as well as allogeneic and autologous stem cell transplantation studies -Aspects of the cell biology, molecular biology, molecular genetics and cytogenetics of normal or diseased hematopoeisis and lymphopoiesis, including stem cells and cytokines and other regulatory systems. Concise, topical review material is welcomed, especially if it makes new concepts and ideas accessible to a wider community. Proposals for review material may be discussed with the Editor-in-Chief. Collections of case material and case reports will be considered only if they have broader scientific or clinical relevance.
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