title: The heterogeneity of pediatric MLL-rearranged acute myeloid leukemia.
abstract: Translocations involving the mixed-lineage leukemia (MLL) gene, localized at 11q23, comprise 15 to 20% of all pediatric acute myeloid leukemia (AML) cases. This review summarizes current knowledge about the etiology, biology, clinical characteristics and differences in outcome in MLL-rearranged pediatric AML. Furthermore, we discuss the role of cooperating events in MLL-rearranged pediatric AML, and future therapeutic strategies to improve outcome. We conclude that MLL-rearranged pediatric AML is a heterogeneous disease, and prognosis depends on various factors, for example, translocation partner, age, WBC and additional cytogenetic aberrations. The relationship of outcome with specific translocation partners requires that they be searched for in the diagnostic work-up of AML. To achieve further improvements in outcome, unraveling the biology of MLL-rearranged pediatric AML is warranted.
authors.affiliation: Department of Pediatric Oncology/Hematology, Erasmus MC/Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: B V
authors.initials: BV
authors.lastname: Balgobind
authors.forename: C M
authors.initials: CM
authors.lastname: Zwaan
authors.forename: R
authors.initials: R
authors.lastname: Pieters
authors.forename: M M
authors.initials: MM
authors.lastname: Van den Heuvel-Eibrink
mesh_terms: D004268:DNA-Binding Proteins / Q000502:physiology; D015321:Gene Rearrangement; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D015470:Leukemia, Myeloid, Acute / Q000188:drug therapy / Q000235:genetics*; D000074008:MDS1 and EVI1 Complex Locus Protein; D051788:Myeloid-Lymphoid Leukemia Protein / Q000235:genetics*; D011379:Prognosis; D011519:Proto-Oncogenes / Q000502:physiology; D059005:Topoisomerase II Inhibitors / Q000627:therapeutic use; D014157:Transcription Factors / Q000502:physiology; D014178:Translocation, Genetic; D051941:fms-Like Tyrosine Kinase 3 / Q000235:genetics
chemical_list: D004268:DNA-Binding Proteins; D000074008:MDS1 and EVI1 Complex Locus Protein; C063575:MECOM protein, human; D059005:Topoisomerase II Inhibitors; D014157:Transcription Factors; D051788:Myeloid-Lymphoid Leukemia Protein; C497970:FLT3 protein, human; D051941:fms-Like Tyrosine Kinase 3