Clinical Analysis of SET-NUP214 Fusion Gene Positive Patients with Acute Leukemia.
10.19746/j.cnki.issn.1009-2137.2023.02.007
- Author:
Yang SONG
1
;
Xiao-Yuan GONG
1
;
Shu-Ning WEI
1
;
Qing-Hua LI
1
;
Guang-Ji ZHANG
1
;
Ying WANG
1
;
Hui WEI
1
;
Dong LIN
1
;
Shang-Zhu LI
1
;
Si-Zhou FENG
1
;
Jian-Xiang WANG
1
;
Ying-Chang MI
2
Author Information
1. Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Tianjin 300020, China.
2. Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College; State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Tianjin 300020, China,E-mail: ychmi@ihcams.ac.cn.
- Publication Type:Journal Article
- Keywords:
SET-NUP214;
acute leukemia;
allo-HSCT;
fusion gene
- MeSH:
Humans;
Adolescent;
Precursor T-Cell Lymphoblastic Leukemia-Lymphoma;
Retrospective Studies;
Leukemia, Myeloid, Acute/therapy*;
Hematopoietic Stem Cell Transplantation;
Acute Disease;
Prognosis;
Leukemia-Lymphoma, Adult T-Cell/therapy*;
Nuclear Pore Complex Proteins
- From:
Journal of Experimental Hematology
2023;31(2):352-357
- CountryChina
- Language:Chinese
-
Abstract:
OBJECTIVE:To analyze the characteristics and prognosis of acute leukemia(AL) with SET-NUP214 fusion gene.
METHODS:The clinical data of 17 patients over 14 years old newly diagnosed with SET-NUP214 positive AL admitted in Institute of Hematology and Blood Diseases Hospital from August 2017 to May 2021 were analyzed retrospectively.
RESULTS:Among the 17 SET-NUP214 positive patients, 13 cases were diagnosed as T-ALL (ETP 3 cases, Pro-T-ALL 6 cases, Pre-T-ALL 3 cases, Medullary-T-ALL 1 case), AML 3 cases (2 cases M5, 1 case M0) and ALAL 1 case. Thirteen patients presented extramedullary infiltration at initial diagnosis. All 17 patients received treatment, and a total of 16 cases achieved complete remission (CR), including 12 cases in patients with T-ALL. The total median OS and RFS time were 23 (3-50) months and 21 (0-48) months, respectively. Eleven patients received allogeneic hematopoietic stem cell transplantation(allo-HSCT), with median OS time of 37.5 (5-50) months and median RFS time of 29.5 (5-48) months. The median OS time of 6 patients in chemotherapy-only group was 10.5 (3-41) months, and median RFS time of 6.5 (3-39) months. The OS and RFS of patients with transplantation group were better than those of chemotherapy-only group (P=0.038). Among the 4 patients who relapsed or refractory after allo-HSCT, the SET-NUP214 fusion gene did not turn negative before transplantation. While, in the group of 7 patients who have not relapsed after allo-HSCT till now, the SET-NUP214 fusion gene expression of 5 patients turned negative before transplantation and other 2 of them were still positive.
CONCLUSION:The fusion site of SET-NUP214 fusion gene is relatively fixed in AL patients, often accompanied by extramedullary infiltration. The chemotherapy effect of this disease is poor, and allo-HSCT may improve its prognosis.
