Monosomy 7/del (7q) in inherited bone marrow failure syndromes: A systematic review. Issue 12 (14th July 2017)

• Record Type: Journal Article
• Authors: Pezeshki, Alex; Podder, Shreya; Kamel, Ralph; Corey, Seth J.
• Is Part Of: Pediatric blood & cancer. Volume 64:Issue 12(2017)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2017-07-14
• Subjects: acute myeloid leukemia -- bone marrow failure syndromes -- monosomy 7; Tumors in children -- Periodicals; Blood -- Diseases -- Periodicals; Cancer in children -- Periodicals; 618.92
• DOI: 10.1002/pbc.26714
• Languages: English
• ISSNs: 1545-5009

Abstract

Abstract: Inherited bone marrow failure syndromes (IBMFS) are rare cancer predisposition syndromes with an especially high risk of transformation to myelodysplastic syndrome (MDS) and/or acute myeloid leukemia (AML). We performed a retrospective systematic review of reported MDS/AML arising in the eight most common IBMFS to determine the frequency and outcome of chromosome 7 abnormalities. We identified 738 MDS/AML cases of 4, 293 individuals. Monosomy 7 or del (7q) occurred in ∼17%. Greater understanding of the roles played by sequential acquisition of genetic and cytogenetic changes will provide insights into myeloid leukemogenesis and improve the surveillance and hopefully outcomes for individuals with IBMFS.