异基因造血干细胞移植治疗12例DEK-NUP214融合基因阳性急性髓系白血病患者的疗效分析
[Analysis of therapeutic effects of allogeneic hematopoietic stem cell transplantation in 12 patients with DEK-NUP214 fusion gene positive acute myeloid leukemia].
发表日期:2024 Apr 14
作者:
Y Y Shen, D L Yang, Y He, A M Pang, X Chen, Q L Ma, R L Zhang, J L Wei, W H Zhai, M Z Han, E L Jiang, S Z Feng
来源:
Experimental Hematology & Oncology
摘要:
该研究纳入2016年11月至2022年8月在中国医学科学院血液病医院接受allo-HSCT治疗的12例DEK-NUP214融合基因阳性急性髓系白血病患者的临床资料,对其临床资料进行回顾性分析。患者包括 5 名男性和 7 名女性,中位年龄为 34 (16-52) 岁。诊断时,所有患者的DEK-NUP214融合基因均呈阳性。染色体核型分析显示10例患者存在t(6;9)(p23;q34)易位(2例患者未进行染色体核型分析),11例患者检测到FLT3-ITD突变,11例患者检测到WT1高表达。 9名患者在移植前原发病处于首次完全缓解状态,1名患者疾病未缓解,2名患者处于复发状态。所有患者均接受清髓预处理,5例患者接受同胞同种异体造血干细胞移植,7例患者接受单倍体造血干细胞移植。移植物中单核细胞的中位数为10.87(7.09-17.89)×10(8)/kg,CD34(+)细胞数为3.29(2.53-6.10)×10(6)/kg。所有患者均实现血液重建,中性粒细胞植入中位时间为14(10-20)天,血小板植入中位时间为15(9-27)天。移植后1年移植相关死亡率为21.2%。移植后1年和3年的累积复发率分别为25.0%和50.0%。无白血病生存率分别为(65.6±14.0)%和(65.6±14.0)%。总生存率分别为(72.2±13.8)%和(72.2±13.8)%。
Twelve DEK-NUP214 fusion gene-positive patients with acute myeloid leukemia and on allo-HSCT treatment at the Hematology Hospital of the Chinese Academy of Medical Sciences from November 2016 to August 2022 were included in the study, and their clinical data were retrospectively analyzed. The patients comprised five men and seven women with a median age of 34 (16-52) years. At the time of diagnosis, all the patients were positive for the DEK-NUP214 fusion gene. Chromosome karyotyping analysis showed t (6;9) (p23;q34) translocation in 10 patients (two patients did not undergo chromosome karyotyping analysis), FLT3-ITD mutation was detected in 11 patients, and high expression of WT1 was observed in 11 patients. Nine patients had their primary disease in the first complete remission state before transplantation, one patient had no disease remission, and two patients were in a recurrent state. All patients received myeloablative pretreatment, five patients received sibling allogeneic hematopoietic stem cell transplantation, and seven patients received haploid hematopoietic stem cell transplantation. The median number of mononuclear cells in the transplant was 10.87 (7.09-17.89) ×10(8)/kg, and the number of CD34(+) cells was 3.29 (2.53-6.10) ×10(6)/kg. All patients achieved blood reconstruction, with a median time of 14 (10-20) days for neutrophil implantation and 15 (9-27) days for platelet implantation. The 1 year transplant-related mortality rate after transplantation was 21.2%. The cumulative recurrence rates 1 and 3 years after transplantation were 25.0% and 50.0%, respectively. The leukemia free survival rates were (65.6±14.0) % and (65.6±14.0) %, respectively. The overall survival rates were (72.2±13.8) % and (72.2±13.8) %, respectively.