title: The biology of pediatric acute megakaryoblastic leukemia.
abstract: Acute megakaryoblastic leukemia (AMKL) comprises between 4% and 15% of newly diagnosed pediatric acute myeloid leukemia patients. AMKL in children with Down syndrome (DS) is characterized by a founding GATA1 mutation that cooperates with trisomy 21, followed by the acquisition of additional somatic mutations. In contrast, non-DS-AMKL is characterized by chimeric oncogenes consisting of genes known to play a role in normal hematopoiesis. CBFA2T3-GLIS2 is the most frequent chimeric oncogene identified to date in this subset of patients and confers a poor prognosis.
authors.affiliation: Department of Oncology and Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN.
authors.forename: Tanja A
authors.initials: TA
authors.lastname: Gruber
authors.affiliation: Department of Pathology, St. Jude Children's Research Hospital, Memphis, TN.
authors.forename: James R
authors.initials: JR
authors.lastname: Downing
mesh_terms: D002648:Child; D006801:Humans; D007947:Leukemia, Megakaryoblastic, Acute / Q000235:genetics* / Q000473:pathology*