Department of Hematology, The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Suzhou, China.
Department of Hematology, Yinzhou People Hospital, Ningbo, China.
Bone Marrow Transplant. 2019 Feb;54(2):236-243. doi: 10.1038/s41409-018-0249-7. Epub 2018 Jun 25.
To investigate the prognosis of patients with adult B-cell acute lymphoblastic leukemia (B-ALL) with Ikaros family zinc-finger 1 (IKZF1) mutation and determine the role of allogeneic hematopoietic stem cell transplantation (allo-HSCT) in improving the clinical outcome, we detected the IKZF1 mutation and BCR-ABL fusion gene at diagnosis in the bone marrow of 164 adult patients with B-ALL, and analyzed the clinical data of these patients retrospectively. Our analysis showed that grade III-IV acute graft-versus-host disease and IKZF1 mutation in the transplantation group and age and IKZF1 mutation in the non-transplantation group were independent factors for poor prognosis by univariate and multivariate analyses.The 3-year overall survival (OS) and leukemia-free survival (LFS) rates were much lower in the IKZF1+/BCR-ABL+ subgroup than in the IKZF1+/BCR-ABL- and IKZF1-/BCR-ABL- subgroups in both the transplantation and non-transplantation groups. The 3-year OS and LFS rates were significantly higher in the transplantation group than in the non-transplantation group with IKZF1 mutation.The study demonstrated that IKZF1 mutation was an independent factor indicating the poor prognosis of adult B-ALL and much worse prognosis in the BCR-ABL+ subgroup in both non-transplantation and transplantation groups. However, allo-HSCT significantly improved the OS and LFS of patients and also their clinical outcomes.
为了探究 Ikaros 家族锌指蛋白 1(IKZF1)突变的成人 B 细胞急性淋巴细胞白血病(B-ALL)患者的预后,并确定异基因造血干细胞移植(allo-HSCT)在改善临床结局方面的作用,我们在 164 例成人 B-ALL 患者的骨髓中检测了 IKZF1 突变和 BCR-ABL 融合基因,并对这些患者的临床数据进行了回顾性分析。我们的分析表明,单因素和多因素分析显示,移植组中急性移植物抗宿主病 3-4 级和 IKZF1 突变以及非移植组中年龄和 IKZF1 突变是不良预后的独立因素。在移植组和非移植组中,IKZF1+/BCR-ABL+亚组的 3 年总生存(OS)和无白血病生存(LFS)率均明显低于 IKZF1+/BCR-ABL-和 IKZF1-/BCR-ABL-亚组。与非移植组相比,移植组 IKZF1 突变患者的 3 年 OS 和 LFS 率明显更高。本研究表明,IKZF1 突变是成人 B-ALL 预后不良的独立因素,在非移植组和移植组中,BCR-ABL+亚组的预后更差。然而,allo-HSCT 显著改善了患者的 OS 和 LFS,也改善了他们的临床结局。