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Zinc Finger Protein 384 (ZNF384) Impact on Childhood Mixed Phenotype Acute Leukemia and B-Cell Precursor Acute Lymphoblastic Leukemia
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  • Tugce Sudutan,
  • Yucel Erbilgin,
  • Ozden Hatirnaz Ng,
  • Zeynep Karakas,
  • Serap Karaman,
  • Fulya Kucukcankurt,
  • Gul Nihal Ozdemir,
  • Tiraje Celkan,
  • Timur Cetin,
  • Sema Aylan Gelen,
  • Şadan Hacısalihoğlu,
  • MÜGE SAYİTOĞLU
Tugce Sudutan
Aziz Sancar Institute of Experimental Medicine, Istanbul University

Corresponding Author:tugcesudutan@gmail.com

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Yucel Erbilgin
Aziz Sancar Institute of Experimental Medicine, Istanbul University
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Ozden Hatirnaz Ng
School of Medicine,Acibadem Mehmet Ali Aydinlar University
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Zeynep Karakas
Istanbul Faculty of Medicine,Istanbul University
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Serap Karaman
Istanbul Faculty of Medicine,Istanbul University
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Fulya Kucukcankurt
Institute of Health Sciences, Istanbul University
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Gul Nihal Ozdemir
Faculty of Medicine, Istinye University
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Tiraje Celkan
Faculty of Medicine, Istinye University
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Timur Cetin
Yeditepe University Hospital
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Sema Aylan Gelen
Faculty of Medicine,Kocaeli University
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Şadan Hacısalihoğlu
Istanbul Kanuni Sultan Suleyman Education and Research Hospital
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MÜGE SAYİTOĞLU
Aziz Sancar Institute of Experimental Medicine, Istanbul University
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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 clinic for risk calculation and treatment decision. ZNF384 rearrangements are one of the new oncogenic subtypes that have been identified in BCP-ALL by recent studies. Also patients with ZNF384 fusions have been reported in with mixed phenotypic acute leukemia (MPAL). In this study, we screened 133 pediatric patients with ALL for the most common ZNF384 fusions; ZNF384-TCF3, ZNF384-EP300 and ZNF384-TAF15 by using qPCR. The total frequency of ZNF384 gene rearrangements was 8.2% in the cohort. We identified ZNF384 fusions in 9.5% of mixed phenotypic leukemia and 7.6% of BCP-ALL groups. Moreover, a novel breakpoint was identified in ZNF384-TCF3 fusion. Patients with MPAL showed significantly higher ZNF384 expression than BCP-ALL and controls. Patients with ZNF384 rearrangements had intermediate survival rates based on other subtypes.
15 Oct 2022Published in Leukemia & Lymphoma volume 63 issue 12 on pages 2931-2939. 10.1080/10428194.2022.2095630