Pathogenesis of ETV6/RUNX1-positive childhood acute lymphoblastic leukemia and mechanisms underlying its relapse

Congcong Sun, Lixian Chang and Xiaofan Zhu _

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Oncotarget. 2017; 8:35445-35459. https://doi.org/10.18632/oncotarget.16367

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Congcong Sun1, Lixian Chang1 and Xiaofan Zhu1

1 Center for Pediatric Blood Disease, State Key Laboratory of Experimental Hematology, Institute of Hematology and Blood Diseases Hospital, Chinese Academy of Medical Sciences, and Peking Union Medical College, Tianjin, P.R. China

Correspondence to:

Xiaofan Zhu, email:

Keywords: ETV6/RUNX1, childhood acute lymphoblastic leukemia, mechanisms, initiation, relapse

Received: November 21, 2016 Accepted: February 23, 2017 Published: March 18, 2017


ETV6/RUNX1 (E/R) is the most common fusion gene in childhood acute lymphoblastic leukemia (ALL). Multiple lines of evidence imply a “two-hit” model for the molecular pathogenesis of E/R-positive ALL, whereby E/R rearrangement is followed by a series of secondary mutations that trigger overt leukemia. The cellular framework in which E/R arises and the maintenance of a pre-leukemic condition by E/R are fundamental to the mechanism that underlies leukemogenesis. Accordingly, a variety of studies have focused on the relationship between the clones giving rise to the primary and recurrent E/R-positive ALL. We review here the most recent insights into the pathogenic mechanisms underlying E/R-positive ALL, as well as the molecular abnormalities prevailing at relapse.

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