Dataset Information

Rare KMT2A-ELL and Novel ZNF56-KMT2A Fusion Genes in Pediatric T-cell Acute Lymphoblastic Leukemia.

ABSTRACT:

Background/aim

Previous reports have associated the KMT2A-ELL fusion gene, generated by t(11;19)(q23;p13.1), with acute myeloid leukemia (AML). We herein report a KMT2A-ELL and a novel ZNF56-KMT2A fusion genes in a pediatric T-lineage acute lymphoblastic leukemia (T-ALL).

Materials and methods

Genetic investigations were performed on bone marrow of a 13-year-old boy diagnosed with T-ALL.

Results

A KMT2A-ELL and a novel ZNF56-KMT2A fusion genes were generated on der(11)t(11;19)(q23;p13.1) and der(19)t(11;19)(q23;p13.1), respectively. Exon 20 of KMT2A fused to exon 2 of ELL in KMT2A-ELL chimeric transcript whereas exon 1 of ZNF56 fused to exon 21 of KMT2A in ZNF56-KMT2A transcript. A literature search revealed four more T-ALL patients carrying a KMT2A-ELL fusion. All of them were males aged 11, 11, 17, and 20 years.

Conclusion

KMT2A-ELL fusion is a rare recurrent genetic event in T-ALL with uncertain prognostic implications. The frequency and impact of ZNF56-KMT2A in T-ALL are unknown.

SUBMITTER: Panagopoulos I

PROVIDER: S-EPMC7943214 | biostudies-literature | 2021 Mar-Apr

REPOSITORIES: biostudies-literature

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Publications

Rare <i>KMT2A-ELL</i> and Novel <i>ZNF56-KMT2A</i> Fusion Genes in Pediatric T-cell Acute Lymphoblastic Leukemia.

Panagopoulos Ioannis I Andersen Kristin K Eilert-Olsen Martine M Rognlien Anne Gro AG Munthe-Kaas Monica Cheng MC Micci Francesca F Heim Sverre S

Cancer genomics & proteomics 20210301 2

<h4>Background/aim</h4>Previous reports have associated the KMT2A-ELL fusion gene, generated by t(11;19)(q23;p13.1), with acute myeloid leukemia (AML). We herein report a KMT2A-ELL and a novel ZNF56-KMT2A fusion genes in a pediatric T-lineage acute lymphoblastic leukemia (T-ALL).<h4>Materials and methods</h4>Genetic investigations were performed on bone marrow of a 13-year-old boy diagnosed with T-ALL.<h4>Results</h4>A KMT2A-ELL and a novel ZNF56-KMT2A fusion genes were generated on der(11)t(11; ...[more]

PMID: 33608309

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