Analysis of the clinical characteristics of 24 cases of hematological malignancies with SET-NUP214 fusion gene
10.3760/cma.j.issn.0253-2727.2021.06.004
- VernacularTitle:SET-NUP214融合基因阳性血液恶性肿瘤24例临床特性分析
- Author:
Shumin CHEN
1
;
Wenjie SONG
;
Yazhen QIN
;
Zheng WANG
;
Hui DANG
;
Yan SHI
;
Qi HE
;
Qian JIANG
;
Hao JIANG
;
Xiaojun HUANG
;
Yueyun LAI
Author Information
1. 北京大学人民医院，北京大学血液病研究所，国家血液系统疾病临床医学研究中心，造血干细胞移植治疗血液病北京市重点实验室 100044
- Keywords:
SET-NUP214 fusion gene;
Leukemia;
Allogeneic hematopoietic stem cell transplantation
- From:
Chinese Journal of Hematology
2021;42(6):459-465
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the expression of SET-NUP214 fusion gene in hematological malignancies and to analyze its related clinical biological characteristics.Methods:The clinical data of 24 patients with SET-NUP214 fusion gene-positive hematological malignancies were retrospectively analyzed, and the Kaplan-Meier method was used for survival analysis.Results:Among the 24 patients with SET-NUP214 fusion gene, 15 cases of acute lymphoblastic leukemia (ALL) (13 cases of T-ALL and 2 cases of B-ALL) , 7 cases of acute myeloid leukemia (AML) , and 2 cases of T/myeloid mixed acute leukemia have been identified. The immunophenotype of 13 cases of T-ALL was mainly characterized by CD3 +CD2 -, 73.3% of ALL was characterized by myeloid marker expression, and 85.7% of AML was characterized by CD7 expression. Complete remission (CR) was achieved in 22 patients (91.7%) after induction chemotherapy. All 24 patients received allogeneic hematopoietic stem cell transplantation (HSCT) . With a median follow-up of 24 months, the 3-year relapse free survival (RFS) of AML and ALL was 85.7% and 33.3%, respectively ( P=0.128) . Comparing 13 cases of SET-NUP214-positive and 62 cases of SET-NUP214-negative T-ALL, the CR rates of induction chemotherapy were 92.3% and 93.5% ( P=0.445) , and the 4-week CR rates of induction chemotherapy were 69.2% and 72.6%, respectively ( P=0.187) ; the differences were not statistically significant. After HSCT, the 3-year RFS of SET-NUP214 +T-ALL and SET-NUP214 -T-ALL was 38.5% and 66.4%, respectively ( P=0.028) , and the difference was statistically significant. Conclusion:The SET-NUP214 fusion gene is mainly detected in T cell-derived hematological malignancies, and the prognosis of SET-NUP214 positive T-ALL is relatively poor.
