The biology, pathogenesis and clinical aspects of acute lymphoblastic leukemia in children with Down syndrome

P. Lee, R. Bhansali, S. Izraeli, N. Hijiya*, J. D. Crispino

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

45 Scopus citations

Abstract

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 Dyrk1a (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.

Original languageEnglish
Pages (from-to)1816-1823
Number of pages8
JournalLeukemia
Volume30
Issue number9
DOIs
StatePublished - 1 Sep 2016

Funding

FundersFunder number
Alpha Omega Alpha Carolyn L Kuckein Student Research Fellowship
Israel Cancer Research Foundation
Rally and Bear Necessities Foundations
National Institutes of Health
National Cancer InstituteR01CA101774
Samuel Waxman Cancer Research Foundation
CHILDREN with CANCER UK
United States-Israel Binational Science Foundation
Israel Science Foundation

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