Fang Qiuyun, Song Yang, Gong Xiaoyuan, Wang Jun, Li Qinghua, Liu Kaiqi, Feng Yahui, Hao Qishan, Li Yan, Wei Hui, Zhang Guangji, Liu Yuntao, Gong Benfa, Wang Ying, Zhou Chunlin, Lin Dong, Liu Bingcheng, Wei Shuning, Gu Runxia, Mi Yingchang, Wang Jianxiang
State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Disease, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, Tianjin, China.
Front Oncol. 2021 Jun 2;11:677034. doi: 10.3389/fonc.2021.677034. eCollection 2021.
Although pediatric-like treatment regimen has remarkably improved the survival rates of adults with acute lymphoblastic leukemia (ALL), the outcome of some adult patients is still poor owing to adverse genetic features. These molecular abnormalities, especially gene deletions, may be considered for the prognosis assessment for adult patients with ALL. In this study, using multiplex ligation-dependent probe amplification (MLPA) method, gene deletions were analyzed in from 211 adult B-ALL patients treated in our center. The data showed that 68.2% (144/211) adult B-ALL patients carried gene deletions, and the frequency is much higher in PhB-ALL patients. gene deletion is the most common gene deletion in adult B-ALL, followed by deletion. In PhB-ALL patients, the overall survival of patients with gene deletions is inferior to that of patients without any gene deletions. More obviously, patients with or deletion had a worse prognosis, whereas, allogeneic hematopoietic stem cell transplantation could improve OS in patients with deletion, but not in patients with CDKN2A/B deletion. Moreover, the outcome of PhB-ALL patients with double deletion of and may be much worse than that of patients with or alone. Minimal residual disease (MRD) was also analyzed together with gene deletions and demonstrated that gene deletions have a negative impact on survival only in MRD positive PhB-ALL patients. In conclusion, gene deletions are closely related with the prognosis of adult PhB-ALL patients.
尽管类似儿童的治疗方案显著提高了成人急性淋巴细胞白血病（ALL）患者的生存率，但由于不良的基因特征，一些成年患者的预后仍然很差。这些分子异常，尤其是基因缺失，可用于评估成年ALL患者的预后。在本研究中，我们使用多重连接依赖探针扩增（MLPA）方法，对在我们中心接受治疗的211例成年B-ALL患者的基因缺失情况进行了分析。数据显示，68.2%（144/211）的成年B-ALL患者存在基因缺失，且在PhB-ALL患者中该频率更高。基因缺失是成年B-ALL中最常见的基因缺失，其次是缺失。在PhB-ALL患者中，存在基因缺失的患者总生存期低于无任何基因缺失的患者。更明显的是，存在或缺失的患者预后更差，而异基因造血干细胞移植可改善存在缺失患者的总生存期，但对存在CDKN2A/B缺失的患者无效。此外，同时存在和缺失的PhB-ALL患者的预后可能比单独存在或缺失的患者更差。我们还将微小残留病（MRD）与基因缺失一起进行了分析，结果表明基因缺失仅对MRD阳性的PhB-ALL患者的生存有负面影响。总之，基因缺失与成年PhB-ALL患者的预后密切相关。
