KMT2A-MAML2 rearrangement emerged and regressed during neuroblastoma therapy without leukemia after 12.8-year follow-up Journal Article
| Authors: | Felix, C. A.; Slater, D. J.; Davenport, J. W.; Yu, X.; Gregory, B. D.; Li, M. M.; Rappaport, E. F.; Cheung, N. K. V. |
| Article Title: | KMT2A-MAML2 rearrangement emerged and regressed during neuroblastoma therapy without leukemia after 12.8-year follow-up |
| 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. © 2021 Wiley Periodicals LLC |
| Keywords: | neuroblastoma; therapy-related leukemia; kmt2a-maml2; top2 poison chemotherapy; top2a cleave-ome |
| Journal Title: | Pediatric Blood and Cancer |
| Volume: | 69 |
| Issue: | 1 |
| ISSN: | 1545-5009 |
| Publisher: | Wiley Periodicals, Inc |
| Date Published: | 2022-01-01 |
| Start Page: | e29344 |
| Language: | English |
| DOI: | 10.1002/pbc.29344 |
| PUBMED: | 34550633 |
| PROVIDER: | scopus |
| PMCID: | PMC9616630 |
| DOI/URL: | |
| Notes: | Article -- Export Date: 3 January 2022 -- Source: Scopus |
