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Ikaros 家族锌指蛋白 1 突变是成人 B 细胞急性淋巴细胞白血病不良预后的独立因素,异基因造血干细胞移植可改善临床结局。

Ikaros family zinc-finger 1 mutation is an independent factor for the poor prognosis of adult B-cell acute lymphoblastic leukemia, and allogeneic hematopoietic stem cell transplantation can improve clinical outcomes.

机构信息

Department of Hematology, The First Affiliated Hospital of Soochow University, Jiangsu Institute of Hematology, Suzhou, China.

Department of Hematology, Yinzhou People Hospital, Ningbo, China.

出版信息

Bone Marrow Transplant. 2019 Feb;54(2):236-243. doi: 10.1038/s41409-018-0249-7. Epub 2018 Jun 25.

Abstract

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.

摘要

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。

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