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International Journal of Hematology
Published in: International Journal of Hematology 4/2016

01-04-2016 | Progress in Hematology

Evolution of myeloid leukemia in children with Down syndrome

Author: Satoshi Saida

Published in: International Journal of Hematology | Issue 4/2016

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Abstract

Children with Down syndrome (DS) have a markedly increased risk of leukemia. They are at particular risk of acute megakaryoblastic leukemia, known as myeloid leukemia associated with DS (ML–DS), the development of which is closely linked to a preceding temporary form of neonatal leukemia called transient abnormal myelopoiesis (TAM). Findings from recent clinical and laboratory studies suggest that constitutional trisomy 21 and GATA1 mutation(s) cause TAM, and that additional genetic alteration(s) including those in epigenetic regulators and signaling molecules are involved in the progression from TAM to ML–DS. Thus, this disease progression represents an important model of multi-step leukemogenesis. The present review focuses on the evolutionary process of TAM to ML–DS, and advances in the understanding of perturbed hematopoiesis in DS with respect to GATA1 mutation and recent findings, including cooperating genetic events, are discussed.
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Metadata
Title
Evolution of myeloid leukemia in children with Down syndrome
Author
Satoshi Saida
Publication date
01-04-2016
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 4/2016
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-016-1959-5

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