Peking University People's Hospital, Peking University Institute of Hematology, National Clinical Research Center for Hematologic Disease, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing, China.
Hematol Oncol. 2024 Jan;42(1):e3251. doi: 10.1002/hon.3251.
Zinc finger protein 384 (ZNF384) rearrangement defined a novel subtype of B-cell acute lymphoblastic leukemia (B-ALL). The prognostic significance of ZNF384 fusion transcript levels represented measurable residual disease remains to be explored. ZNF384 fusions were screened out in 57 adult B-ALL patients at diagnosis by real-time quantitative polymerase chain reaction and their transcript levels were serially monitored during treatment. The reduction of ZNF384 fusion transcript levels at the time of achieving complete remission had no significant impact on survival, whereas its ≥2.5-log reduction were significantly associated with higher relapse free survival (RFS) and overall survival (OS) rates after course 1 consolidation (p = 0.022 and = 0.0083) and course 2 consolidation (p = 0.0025 and = 0.0008). Compared with chemotherapy alone, allogeneic hematopoietic stem cell transplantation (allo-HSCT) significantly improved RFS and OS of patients with <2.5-log reduction after course 1 consolidation (p < 0.0001 and = 0.0002) and course 2 consolidation (p = 0.0003 and = 0.019), whereas exerted no significant effects in patients with ≥2.5-log reduction (all p > 0.05). ZNF384 fusion transcript levels after course 1 and course 2 consolidation strongly predict relapse and survival and may guide whether receiving allo-HSCT in adult B-ALL.
锌指蛋白 384（ZNF384）重排定义了一种新的 B 细胞急性淋巴细胞白血病（B-ALL）亚型。ZNF384 融合转录本水平代表可测量残留疾病的预后意义仍有待探索。通过实时定量聚合酶链反应在 57 例成人 B-ALL 患者诊断时筛选出 ZNF384 融合，并在治疗过程中连续监测其转录本水平。在达到完全缓解时 ZNF384 融合转录本水平的降低对生存没有显著影响，而其≥2.5-log 降低与第 1 轮巩固治疗后无复发生存率（RFS）和总生存率（OS）显著相关（p=0.022 和 p=0.0083）和第 2 轮巩固治疗（p=0.0025 和 p=0.0008）。与单独化疗相比，异基因造血干细胞移植（allo-HSCT）显著改善了第 1 轮巩固治疗后融合转录本水平<2.5-log 降低的患者的 RFS 和 OS（p<0.0001 和 p=0.0002）和第 2 轮巩固治疗（p=0.0003 和 p=0.019），而在融合转录本水平≥2.5-log 降低的患者中无显著影响（均 p>0.05）。第 1 轮和第 2 轮巩固治疗后 ZNF384 融合转录本水平强烈预测复发和生存，可能指导成人 B-ALL 是否接受 allo-HSCT。
