Zinc finger protein 384 (ZNF384) impact on childhood mixed phenotype acute leukemia and B-cell precursor acute lymphoblastic leukemia

Sudutan T., Erbilgin Y., HATIRNAZ NG Ö., Karaman S., Karakas Z., Kucukcankurt F., ...More

LEUKEMIA & LYMPHOMA, vol.63, no.12, pp.2931-2939, 2022 (SCI-Expanded, Scopus) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 63 Issue: 12
  • Publication Date: 2022
  • Doi Number: 10.1080/10428194.2022.2095630
  • Journal Name: LEUKEMIA & LYMPHOMA
  • Journal Indexes: Science Citation Index Expanded (SCI-EXPANDED), Scopus, Academic Search Premier, CAB Abstracts, EMBASE, MEDLINE, Veterinary Science Database
  • Page Numbers: pp.2931-2939
  • Keywords: B-cell precursor ALL, mixed phenotype acute leukemia, ZNF384 fusion, ZNF384 expression levels, GENE-EXPRESSION, CLASSIFICATION, ABNORMALITIES, REARRANGEMENT, SUBTYPE, TCF3, E2A
  • Kocaeli University Affiliated: Yes

Abstract

B-cell precursor acute lymphoblastic leukemia (BCP-ALL) is a heterogeneous malignancy and consists of several genetic abnormalities. Some of these abnormalities are used in clinics for risk calculation and treatment decisions. Patients with ZNF384 rearrangements had a distinct expression profile regardless of their diagnosis, BCP-ALL or mixed phenotype acute leukemia (MPAL) and defined as a new subtype of ALL. In this study, we screened 42 MPAL and 91 BCP-ALL patients for the most common ZNF384 fusions; ZNF384::TCF3, ZNF384::EP300 and ZNF384::TAF15 by using PCR. We identified ZNF384 fusions in 9.5% of MPAL and 7.6% of BCP-ALL. A novel breakpoint was identified in ZNF384::TCF3 fusion in one BCP-ALL patient. T-myeloid MPAL patients showed significantly lower ZNF384 expression compared to lymphoid groups. Patients with ZNF384r had intermediate survival rates based on other subtypes. Prognostic and patient-specific treatment evaluation of ZNF384 fusions in both ALL and MPAL might help to improve risk characterization of patients.