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
,
2
;
Xiao-Yuan GONG
1
,
2
;
Shu-Ning WEI
1
,
2
;
Qing-Hua LI
1
,
2
;
Guang-Ji ZHANG
1
,
2
;
Ying WANG
1
,
2
;
Hui WEI
1
,
2
;
Dong LIN
1
,
2
;
Shang-Zhu LI
1
,
2
;
Si-Zhou FENG
1
,
2
;
Jian-Xiang WANG
1
,
2
;
Ying-Chang MI
1
,
3
Author Information
1. Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College
2. State Key Laboratory of Experimental Hematology, National Clinical Research Center for Blood Diseases, Tianjin 300020, China.
3. 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.
