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Med Sci (Paris). 2018 Nov;34(11):954-962. doi: 10.1051/medsci/2018237. Epub 2018 Dec 10.

[Pediatric de novo acute megakaryoblastic leukemia: an affair of complexes].

[Article in French]

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Inserm U1170, Institut Gustave Roussy, Pavillon recherche 2, 39 rue Camille Desmoulins, 94800 Villejuif, France.


Pediatric acute megakaryoblastic leukemia (AMKL) are generally associated with poor prognosis and the expression of fusion oncogenes involving transcriptional regulators. Recent results indicate that the ETO2-GLIS2 fusion, associated with 25-30 % of pediatric AMKL, binds and alters the activity of regulatory regions of gene expression, called "enhancers", resulting in the deregulation of GATA and ETS factors essential for the development of hematopoietic stem cells. An imbalance in GATA/ETS factor activity is also found in other AMKL subgroups. This review addresses the transcriptional bases of transformation in pediatric AMKL and therapeutic perspectives.


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