KMT2A‐MAML2 rearrangement emerged and regressed during neuroblastoma therapy without leukemia after 12.8‐year follow‐up. Issue 1 (22nd September 2021)

• Record Type: Journal Article
• Authors: Felix, Carolyn A.; Slater, Diana J.; Davenport, James W.; Yu, Xiang; Gregory, Brian D.; Li, Marilyn M.; Rappaport, Eric F.; Cheung, Nai‐Kong V.
• Is Part Of: Pediatric blood & cancer. Volume 69:Issue 1(2022)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2021-09-22
• Subjects: KMT2A‐MAML2 -- neuroblastoma -- therapy‐related leukemia -- TOP2 poison chemotherapy -- TOP2A cleave‐ome; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.29344
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Twelvepatients without therapy‐related leukemia were studied after completing TOP2 poison chemotherapy in a high‐risk neuroblastoma regimen. One patient harbored an inv(11) that was a KMT2A rearrangement. The KMT2A‐MAML2 transcript was expressed at low level. The patient was prospectively followed. The inv(11) was undetectable in ensuing samples. Leukemia never developed after a 12.8‐year follow‐up period. Enriched etoposide‐induced TOP2A cleavage in the relevant MAML2 genomic region supports a TOP2A DNA damage mechanism. After completing TOP2 poison chemotherapies, covert KMT2A ‐R clones may occur in a small minority of patients; however, not all KMT2A rearrangements herald a therapy‐related leukemia diagnosis.