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

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 withZNF384rearrangements had a distinct expres- sion 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 commonZNF384fusions; ZNF384::TCF3, ZNF384::EP300andZNF384::TAF15by using PCR. We identifiedZNF384fusions in 9.5% of MPAL and 7.6% of BCP-ALL. A novel breakpoint was identified inZNF384::TCF3fusion in one BCP-ALL patient. T-myeloid MPAL patients showed significantly lowerZNF384expression compared to lymphoid groups. Patients withZNF384r had intermediate survival rates based on other subtypes. Prognostic and patient- specific treatment evaluation ofZNF384fusions in both ALL and MPAL might help to improve risk characterization of patients.