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

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 reg...

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Bibliographic Details
Published in:Leukemia & lymphoma 2022-10, Vol.63 (12), p.2931-2939
Main Authors: Sudutan, Tugce, Erbilgin, Yucel, Hatirnaz Ng, Ozden, Karaman, Serap, Karakas, Zeynep, Kucukcankurt, Fulya, Celkan, Tiraje, Timur, Cetin, Ozdemir, Gul Nihal, Hacısalihoglu, Sadan, Gelen, Sema Aylan, Sayitoğlu, Müge
Format: Article
Language:English
Subjects:
B-cell precursor ALL
Burkitt Lymphoma
Child
Humans
mixed phenotype acute leukemia
Phenotype
Precursor Cell Lymphoblastic Leukemia-Lymphoma - diagnosis
Precursor Cell Lymphoblastic Leukemia-Lymphoma - genetics
Precursor Cell Lymphoblastic Leukemia-Lymphoma - pathology
Trans-Activators - genetics
Trans-Activators - metabolism
Transcription Factors - genetics
Zinc Fingers
ZNF384 expression levels
ZNF384 fusion
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Summary: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.
ISSN:1042-8194
1029-2403
DOI:10.1080/10428194.2022.2095630