The changing scenario of non-Down syndrome acute megakaryoblastic leukemia in children

Riccardo Masetti, Vanessa Guidi, Laura Ronchini, Nicola Salvatore Bertuccio, Franco Locatelli, Andrea Pession

Risultato della ricerca: Contributo in rivistaArticolo in rivista

Abstract

Pediatric non-Down-syndrome acute megakaryoblastic leukemia (non-DS-AMKL) is a heterogeneous subtype of leukemia that has historically been associated with poor prognosis. Until the advent of large-scale genomic sequencing, the management of patients with non-DS-AMKL was very difficult due to the absence of reliable biological prognostic markers. The sequencing of large cohort of pediatric non-DS-AMKL samples led to the discovery of novel genetic aberrations, including high-frequency fusions, such as CBFA2T3-GLIS2 and NUP98-KDM5 A, as well as less frequent aberrations, such as HOX rearrangements. These new insights into the genetic landscape of pediatric non-DS-AMKL has allowed refining the risk-group stratification, leading to important changes in the prognostic scenario of these patients. This review summarizes the most important molecular pathogenic mechanisms of pediatric non-DS-AMKL. A critical discussion on how novel genetic abnormalities have refined the risk profile assessment and changed the management of these patients in clinical practice is also provided.
Lingua originaleEnglish
pagine (da-a)132-138
Numero di pagine7
RivistaCRITICAL REVIEWS IN ONCOLOGY HEMATOLOGY
Volume138
DOI
Stato di pubblicazionePubblicato - 2019

Keywords

  • Acute megakaryoblastic leukemia
  • FAB M7
  • AMKL

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