Tan Zhengwei, Zhang Xinhe, Feng Jia, Zhao Yuechao, Hu Huijin, Wu Dijiong, Yu Qinghong, Zhang Yu, Wu Liqiang, Hu Tonglin, Yan Zhengsong, Ye Baodong, Liu Wenbin
The First School of Clinical Medicine, Zhejiang Chinese Medical University, Hangzhou, China.
Department of Hematology, The First Affiliated Hospital of Zhejiang Chinese Medical University (Zhejiang Provincial Hospital of Traditional Chinese Medicine), Hangzhou, China.
Ann Hematol. 2025 Mar;104(3):1877-1886. doi: 10.1007/s00277-024-06152-6. Epub 2025 Jan 28.
Aplastic anemia (AA) is a life-threatening bone marrow failure syndrome. The advent of next-generation sequencing (NGS) has shed light on the link between somatic mutations (SM) and the efficacy of immunosuppressive therapy (IST) in AA patients. However, the relationship between SM and hematopoietic stem cell transplantation (HSCT) has not been extensively explored. In this retrospective analysis, we examined 166 AA patients who received HSCT or IST at our institution between May 2019 and December 2023. NGS was conducted on 66 genes within bone marrow cells to investigate the correlation between SM and the prognosis and therapeutic response in AA patients, as well as to assess the impact of mutation types on HSCT outcomes. Clinical data were gathered from 166 AA patients, comprising 84 males and 82 females, with a median age of 32 years (ranging from 9 to 75 years). In our study, a total of 151 somatic mutations were identified across 84 patients (50.6%), with 42 patients (25.3%) presenting a single mutation and 26 patients (15.7%) harboring two mutations. The top five genes with the highest mutation frequency were BCOR/BCORL1 (12.6%), ASXL1 (8.6%), TET2 (6.6%), CEBPA (5.3%), and GATA2 (4.6%). We stratified patients into SM and No-SM groups based on the presence of mutations and further divided them into HSCT and IST groups to assess the influence of mutation types on treatment response and survival within and between these groups. The findings were as follows: 1.Patients in the HSCT group exhibited a higher treatment response (OR 85.9% vs. 68.4%, p < 0.05), although there was no significant difference in survival. 2.Patients with favorable mutations, such as PIGA and BCOR/BCORL1, experienced significantly improved response and survival compared to those with unfavorable mutations like ASXL1, DNMT3A, and TET2 (OR 93.7% vs. 72%, p < 0.05) (3-year OS 93.7% vs. 80%, p > 0.05). 3.The HSCT-Favorable group demonstrated superior response rates (OR 100% vs. 67.7%, p < 0.05) and longer survival (3-year OS 100% vs. 67.7%, p < 0.05) compared to the IST-Favorable group. This study underscores that AA patients carrying favorable mutations, particularly BCOR/BCORL1, tend to have a more robust response and better prognosis than those without mutations or those with unfavorable mutations, such as ASXL1/DNMT3A. These findings are especially pertinent to HSCT, highlighting the importance of NGS prior to initiating treatment.
再生障碍性贫血(AA)是一种危及生命的骨髓衰竭综合征。新一代测序(NGS)技术的出现揭示了体细胞突变(SM)与AA患者免疫抑制治疗(IST)疗效之间的联系。然而,SM与造血干细胞移植(HSCT)之间的关系尚未得到广泛研究。在这项回顾性分析中,我们研究了2019年5月至2023年12月期间在我们机构接受HSCT或IST治疗的166例AA患者。对骨髓细胞中的66个基因进行了NGS检测,以研究SM与AA患者预后和治疗反应之间的相关性,并评估突变类型对HSCT结果的影响。收集了166例AA患者的临床数据,其中男性84例,女性82例,中位年龄为32岁(9至75岁)。在我们的研究中,共在84例患者(50.6%)中鉴定出151个体细胞突变,其中42例患者(25.3%)为单基因突变,26例患者(15.7%)为双基因突变。突变频率最高的前五个基因是BCOR/BCORL1(12.6%)、ASXL1(8.6%)、TET2(6.6%)、CEBPA(5.3%)和GATA2(4.6%)。我们根据是否存在突变将患者分为SM组和无SM组,并进一步分为HSCT组和IST组,以评估突变类型对这些组内和组间治疗反应和生存的影响。结果如下:1.HSCT组患者的治疗反应更高(OR 85.9%对68.4%,p<0.05),尽管生存率无显著差异。2.与携带ASXL1、DNMT3A和TET2等不良突变的患者相比,携带PIGA和BCOR/BCORL1等有利突变的患者反应和生存率显著提高(OR 93.7%对72%,p<0.05)(3年总生存率93.7%对80%,p>0.05)。3.HSCT-有利组的反应率高于IST-有利组(OR 100%对67.7%,p<0.05),生存率更长(3年总生存率100%对67.7%,p<0.05)。这项研究强调,携带有利突变,特别是BCOR/BCORL1的AA患者,比没有突变或携带ASXL1/DNMT3A等不良突变的患者往往反应更强,预后更好。这些发现对于HSCT尤其相关,突出了在开始治疗前进行NGS的重要性。