[Analysis of the clinical characteristics of 24 cases of hematological malignancies with SET-NUP214 fusion gene].

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.

• Authors: S M Chen, W J Song, Y Z Qin, Z Wang, H Dang, Y Shi, Q He, Q Jiang, H Jiang, X J Huang, Y Y Lai
• Journal: Zhonghua xue ye xue za zhi = Zhonghua xueyexue zazhi (Zhonghua Xue Ye Xue Za Zhi) Vol. 42 Issue 6 Pg. 459-465 (Jun 14 2021) ISSN: 0253-2727 [Print] China
• PMID: 34384151 (Publication Type: Journal Article)

Chemical References

• DNA-Binding Proteins
• Histone Chaperones
• NUP214 protein, human
• Nuclear Pore Complex Proteins
• SET protein, human

Topics

• DNA-Binding Proteins
• Hematologic Neoplasms (genetics)
• Hematopoietic Stem Cell Transplantation
• Histone Chaperones
• Humans
• Leukemia, Myeloid, Acute
• Nuclear Pore Complex Proteins
• Precursor Cell Lymphoblastic Leukemia-Lymphoma (genetics)
• Precursor T-Cell Lymphoblastic Leukemia-Lymphoma
• Prognosis
• Retrospective Studies