Adult acute T-lymphocytic leukemia patients with SET-NUP214 fusion gene: report of four cases and review of literature
10.3760/cma.j.issn.1009-9921.2019.03.007
- VernacularTitle:伴SET-NUP214融合基因的成年人急性T淋巴细胞白血病四例并文献复习
- Author:
Jian ZHOU
1
;
Fanli LIN
;
Hengwei LIU
;
Lin ZHENG
;
Chunlan HUANG
Author Information
1. 成都市第三人民医院血液科 610031
- Keywords:
Leukemia,lymphoid;
SET-NUP214 fusion gene;
Polymerase chain reaction;
Minimal residual disease
- From:
Journal of Leukemia & Lymphoma
2019;28(3):155-159
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical and biological characteristics of adult acute T-lymphocytic leukemia (T-ALL) patients carrying SET-NUP214 fusion gene,and the prognostic value of SET-NUP214 molecular marker monitoring.Methods The clinical and laboratory data of 4 adult T-ALL patients with SET-NUP214 fusion gene in the Third People's Hospital of Chengdu from January 2009 to December 2014 were analyzed retrospectively,and T cell receptor (TCR) gene rearrangement was detected to judge the differentiation and developmental stages of tumor cells in these patients.Minimal residual disease (MRD) was detected in 2 patients with follow-up specimens through detection of SET-NUP214 gene by using polymerase chain reaction.Results Four patients expressed T cell immune markers CD5,CD7,and cytoplasmic CD3 (cyCD3),and also expressed some myeloid-specific antigens.All 4 patients had the same SET-NUP214 fusion site.In the tumor cells of 4 patients,5 TCRB gene rearrangements were detected,all of which were incomplete rearrangement of DB-JB;4 patients were detected with TCRG and TCRD gene rearrangements,and all were completely rearranged.The result of MRD monitoring through SET-NUP214 fusion gene was consistent with clinical treatment outcome.Conclusions The T-ALL patients with SET-NUP214 fusion gene have some unique cell biological characteristics.SET-NUP214 fusion gene could be used as a molecular marker for MRD monitoring,and which can be used for the follow-up in the course of treatment.
