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. 2023 Mar 16;23(2):298-309.
doi: 10.17305/bjbms.2022.7851.

Clinical features and outcomes of fusion gene defined adult Ph-negative B-cell precursor acute lymphoblastic leukemia patients: A single institutional report

Kai Sun  1 , Jun Wang  1 , Wen-Min Chen  1 , Nan Xu  1 , Ling-Yu Long  1 , Xu Wang  1 , Hao Jiang  1 , Qian Jiang  1 , Xiao-Jun Huang  1 , Ya-Zhen Qin  1
Affiliations

Clinical features and outcomes of fusion gene defined adult Ph-negative B-cell precursor acute lymphoblastic leukemia patients: A single institutional report

Kai Sun et al. Biomol Biomed. .

Abstract

More clinical studies are needed to clarify the risk stratification by the integration of all fusion genes in adult B-cell precursor acute lymphoblastic leukemia (BCP-ALL). A total of 320 consecutive adult Ph-negative BCP-ALL patients who had been tested classical fusions (KMT2A rearrangement and TCF3-PBX1) at diagnosis were further retrospectively screened novel fusion genes (Ph-like, ZNF384 and MEF2D fusions) by multiplex real-time quantitative PCR (RQ-PCR). Classical fusions were identified in 12.5% of patients, while 4.4%, 17.2% and 3.8% of patients were identified Ph-like, ZNF384 and MEF2D fusions, respectively. 1-course CR rate, relapse-free survival (RFS) and overall survival (OS) rates tended to show or showed statistically significant differences among fusion-defined subgroups (P = 0.084, 0.001 and 0.0093, respectively). Based on individual outcomes, patients with KMT2A rearrangement, TCF3-PBX1, Ph-like, and MEF2D fusions were classified into fusion-defined high-risk group (n = 66, 20.6%). High-risk group had significantly lower 3-year RFS and 3-year OS rates than standard-risk group (P 0.001 and = 0.0022), and was an independent adverse prognostic factor for RFS in the entire cohort (P 0.001). In conclusion, the spectrum of fusion genes in the current Chinese cohort was distinct from that in reports from western countries. Detection of fusion genes improved risk stratification in adult Ph-negative BCP-ALL patients.

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Conflict of interest statement

Conflicts of interest: The authors declare no conflicts of interest.

Figures

Figure 1.
Figure 1.
The expression pattern of P2RY8-CRLF2. (A) Expression pattern of P2RY8-CRLF2 in 290 patients; (B) Expression pattern of P2RY8-CRLF2 in patients grouped by fusion types with detectable numbers and proportion in fusion-defined groups shown above each graph.
Figure 2.
Figure 2.
Distribution of fusion-defined patients.
Figure 3.
Figure 3.
RFS and OS in fusion-defined groups. (A) RFS in the whole cohort; (B) OS in the whole cohort; (C) RFS of patients who received allo-HSCT and were censored at the time of transplantation; (D) OS of patients who received allo-HSCT and were censored at the time of transplantation. RFS: Relapse-free survival; OS: Overall survival; allo-HSCT: Allogeneic hematopoietic stem cell transplantation.
Figure 4.
Figure 4.
RFS and OS in fusion-defined risk. (A) RFS in the whole cohort; (B) OS in the whole cohort; (C) RFS of patients who received allo-HSCT and were censored at the time of transplantation; (D) OS of patients who received allo-HSCT and were censored at the time of transplantation. RFS: Relapse-free survival; OS: Overall survival; allo-HSCT: Allogeneic hematopoietic stem cell transplantation.
See this image and copyright information in PMC

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