Increased Leukemia Risk in Children With Down Syndrome

Abstract

Research Article| October 01 2021 Increased Leukemia Risk in Children With Down Syndrome AAP Grand Rounds (2021) 46 (4): 45. https://doi.org/10.1542/gr.46-4-45 Views Icon Views Article contents Figures & tables Video Audio Supplementary Data Peer Review Share Icon Share Twitter LinkedIn Tools Icon Tools Get Permissions Cite Icon Cite Search Site Citation Increased Leukemia Risk in Children With Down Syndrome. AAP Grand Rounds October 2021; 46 (4): 45. https://doi.org/10.1542/gr.46-4-45 Download citation file: Ris (Zotero) Reference Manager EasyBib Bookends Mendeley Papers EndNote RefWorks BibTex toolbar search nav search search input Search input auto suggest search filter All PublicationsAll JournalsAAP Grand RoundsPediatricsHospital PediatricsPediatrics In ReviewNeoReviewsAAP NewsAll AAP Sites Search Advanced Search Topics: down syndrome, leukemia Source: Marlow EC, Ducore J, Kwan ML, et al Leukemia risk in a cohort of 3.9 million children with and without Down syndrome. J Pediatr. 2021;234:172-180.e3; doi:10.1016/j.jpeds.2021.03.001Google Scholar Investigators from multiple institutions conducted a retrospective cohort study to assess leukemia risks in children with Down syndrome. For the study, they included data on all live births at 7 US healthcare systems and in Ontario, Canada, between 1996 and 2016. Children with Down syndrome were identified by ICD-9 and ICD-10 codes. Cancer diagnoses were obtained from tumor registries in the US and the Pediatric Oncology Group of Ontario Networked Information System. Additional information collected included sex, age, and maternal age at birth; race and ethnicity data were available on US children. Study participants were followed until age 15, any cancer diagnosis, death, or disenrollment from the healthcare system. The primary study outcome was incident diagnosis of primary leukemia and incident diagnosis of specific leukemias including acute lymphoblastic leukemia (ALL), acute myeloid leukemia (AML), and megakaryoblastic leukemia (AML-M7) based on the 2008 WHO classification. The incidence of leukemia (overall and by subtype) in children with Down syndrome and those without was compared using Cox proportional hazard models, with analyses stratified by age (<5 and ≥5 years old). Data were analyzed on 3,905,399 children, including 4,401 with Down syndrome (incidence 1.12/1,000 children). Study participants with Down syndrome were more likely to be male (54.7% vs 51.3% in those without Down syndrome), born to mothers ≥40 years old (18.6% vs 3.7%), and in the US cohort more likely to be white (73.1% vs 65.0%) and of Hispanic ethnicity (24.8% vs 19.3%). Among children with Down syndrome, the incidence of AML peaked in the first year of life (incidence 619.4 per 100,000), with ALL becoming more common in those 2 to 4 years old (incidence ranging from 333.3 to 326.8/100,000). For other children, the incidence of AML was low at all ages (≤1.1/100,000); the incidence of ALL peaked at age 3 years (11.2/100,000). Down syndrome was associated with an increased risk for any leukemia (hazard ratio [HR], 75.6; 95% CI, 62.3, 91.6 for those <5 years old; HR, 18.3; 95% CI, 7.8, 43.1 for those ≥5 years old). Among those <5 years old, Down syndrome was associated with an increased risk of AML-M7 (HR, 1,500; 95% CI, 555, 4,070) and other AML (HR, 197; 95% CI, 70, 553). Children with Down syndrome also were at increased risk for ALL (HR, 28.4; 95% CI, 20.4, 39.6 for those <5 years old; HR, 21.4; 95% CI, 12.1, 37.9 for those ≥5 years old). The authors conclude that Down syndrome is a strong risk factor for childhood leukemia. Dr Hogan has disclosed no financial relationship relevant to this commentary. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device. Down syndrome (DS), arising in an estimated 1 in 800 to 1,000 live births in the US annually,1 is... You do not currently have access to this content.