The Biology, Pathogenesis and Clinical Aspects of Acute Lymphoblastic Leukemia in Children with Down Syndrome

Overview

Journal Leukemia

Specialties Hematology
Oncology

Date 2016 Jun 11

PMID 27285583

Citations 29

Authors

P Lee

R Bhansali

S Izraeli

N Hijiya

J D Crispino

Affiliations

Soon will be listed here.

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.

Citing Articles

Impaired neutrophil-mediated cell death drives Ewing's Sarcoma in the background of Down syndrome.

Peirone S, Tirtei E, Campello A, Parlato C, Guarrera S, Mareschi K Front Oncol. 2024; 14:1429833.

PMID: 39421445 PMC: 11484044. DOI: 10.3389/fonc.2024.1429833.

Down syndrome-associated leukaemias: current evidence and challenges.

Mason N, Cahill H, Diamond Y, McCleary K, Kotecha R, Marshall G Ther Adv Hematol. 2024; 15:20406207241257901.

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Insights from the protein interaction Universe of the multifunctional "Goldilocks" kinase DYRK1A.

Ananthapadmanabhan V, Shows K, Dickinson A, Litovchick L Front Cell Dev Biol. 2023; 11:1277537.

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Hematologic Neoplasms Associated with Down Syndrome: Cellular and Molecular Heterogeneity of the Diseases.

Peroni E, Gottardi M, DAntona L, Randi M, Rosato A, Coltro G Int J Mol Sci. 2023; 24(20).

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Discovery and Synthesis of a Naturally Derived Protein Kinase Inhibitor that Selectively Inhibits Distinct Classes of Serine/Threonine Kinases.

Du L, Wilson B, Li N, Shah R, Dalilian M, Wang D J Nat Prod. 2023; 86(10):2283-2293.

PMID: 37843072 PMC: 10616853. DOI: 10.1021/acs.jnatprod.3c00394.

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