AUTHOR=Qin Ya-Zhen , Jiang Qian , Xu Lan-Ping , Wang Yu , Jiang Hao , Dao Feng-Ting , Chen Wen-Min , Zhao Xiao-Su , Liu Yan-Rong , Zhang Xiao-Hui , Liu Kai-Yan , Huang Xiao-Jun 

TITLE=The Prognostic Significance of ZNF384 Fusions in Adult Ph-Negative B-Cell Precursor Acute Lymphoblastic Leukemia: A Comprehensive Cohort Study From a Single Chinese Center

JOURNAL=Frontiers in Oncology

VOLUME=11

YEAR=2021

URL=https://www.frontiersin.org/journals/oncology/articles/10.3389/fonc.2021.632532

DOI=10.3389/fonc.2021.632532

ISSN=2234-943X

ABSTRACT=<p>Novel recurrent fusion gene types such as zinc finger protein 384 (<italic>ZNF384</italic>) fusions have been identified in B-cell precursor acute lymphoblastic leukemia (BCP-ALL) with the application of next-generation sequencing technologies. However, the comprehensive large-scale clinical cohort study for clarifying their prognostic significance remains scarce to date. A total of 242 consecutive adult Ph-negative BCP-ALL patients treated in our institute were retrospectively screened <italic>ZNF384</italic> fusions at diagnosis by multiplex real time quantitative PCR. <italic>ZNF384</italic> fusions were identified in 47 patients (19.4%) and all belonged to B-other ALL (having no high hyperdiploid karyotype, <italic>BCR</italic>-<italic>ABL1</italic>, <italic>TCF3</italic>-<italic>PBX1</italic>, <italic>ETV6</italic>-<italic>RUNX1</italic>, or <italic>MLL</italic> rearrangement). In the whole cohort, patients with <italic>ZNF384</italic> fusions had significantly higher 3-year relapse-free-survival (RFS) and tended to have a higher 3-year overall survival (OS) than those with no <italic>ZNF384</italic> fusions (80.1% <italic>vs.</italic> 52.5%, <italic>P</italic> = 0.013; 67.6% <italic>vs.</italic> 54.0%, <italic>P</italic> = 0.10). For patients receiving chemotherapy alone and received allogeneic-hematologic stem cell transplantation (allo-HSCT) were censored at the time of transplantation, patients with <italic>ZNF384</italic> fusions had both similar RFS and similar OS to B-other ALL patients with no <italic>ZNF384</italic> fusions (RFS: P =0.94 and 0.30; OS: P =0.94 and 0.51). For patients receiving transplantation, those with <italic>ZNF384</italic> fusions had significantly higher 3-year RFS than B-other ALL patients with no <italic>ZNF384</italic> fusions and their OS were similar (P = 0.022 and 0.24). Only two of 31 patients with <italic>ZNF384</italic> fusions and receiving allo-HSCT relapsed, individually occurred 66.8 and 69.8 months after transplantation. Therefore, <italic>ZNF384</italic> fusion is common in adult BCP-ALL, which may define a new group from BCP-ALL containing no classical fusion transcript with better prognosis through receiving allo-HSCT.</p>