Journal
LEUKEMIA
Volume 30, Issue 9, Pages 1816-1823Publisher
NATURE PUBLISHING GROUP
DOI: 10.1038/leu.2016.164
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Funding
- National Institutes of Health [R01 CA101774]
- Rally and Bear Necessities Foundations
- Unites States-Israel Binational Science Foundation
- Samuel Waxman Cancer Research Foundation
- Israel Science Foundation Legacy Program
- Israel Cancer Research Foundation
- Children with Cancer UK
- Alpha Omega Alpha Carolyn L Kuckein Student Research Fellowship
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Children with Down syndrome (DS) are at a 20-fold increased risk for acute lymphoblastic leukemia (DS-ALL). Although the etiology of this higher risk of developing leukemia remains largely unclear, the recent identification of CRLF2 (cytokine receptor like factor 2) and JAK2 mutations and study of the effect of trisomy of Hmgn1 and Dyrk1 alpha (dual-specificity tyrosine phosphorylation-regulated kinase 1A) on B-cell development have shed significant new light on the disease process. Here we focus on the clinical features, biology and genetics of ALL in children with DS. We review the unique characteristics of DS-ALL on both the clinical and molecular levels and discuss the differences in treatments and outcomes in ALL in children with DS compared with those without DS. The identification of new biological insights is expected to pave the way for novel targeted therapies.
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