title: [Genetic prognostic factors in childhood acute myeloid leukemia].
abstract: The survival rate of children and adolescents suffering acute myeloid leukemia (AML) has been significantly improved within the last decades. This has been achieved by a continuously intensified therapy and progress in supportive care to prevent and treat complications. In Germany, the AML-BFM trials 98 (n=413) and 2004 (n=499) enrolled 912 children and adolescents as protocol patients (1998-2010). The 5-year-overall survival was 71±2%. In the previous studies prognosis and subsequent treatment stratification based on morphology, cytochemistry and white blood cell count. Today, the identification of new genetic aberrations in AML enables a genetically determined estimation of prognosis, although treatment response must be considered for treatment stratification. The group with a favorable prognosis summarized AML with t(8;21), inv(16), t(15;17), t(1;11), and AML with normal karyotype and NPM1-mutation (n=253; EFS 74±3%, OS 88±2%). A poor prognosis (HR-group) must be expected in AML with t(4;11), t(5;11), t(6;11), t(6;9), t(7;12), t(9;22), Monosomy 7, combined FLT3/WT1-mutation, and AML with der(12p)-aberration (n=101; EFS 30±5%; OS 56±5%). The intermediate group summarizes all other subgroups especially AML with normal karyotyp, AML with FLT3-ITD or t(9;11) (n=558; EFS 43±2%; OS 64±2%). The validation of the internationally identified, genetically determined prognostic factors within the AML-BFM (Germany) study population will support treatment recommendations.
authors: Pädiatrische Onkologie und Hämatologie, Medizinische Hochschule Hannover. reinhardt.dirk@mh-hannover.de
authors: D
authors: D
authors: Reinhardt
authors: C
authors: C
authors: Von Neuhoff
authors: A
authors: A
authors: Sander
authors: U
authors: U
authors: Creutzig
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations; D002872:Chromosome Deletion; D025063:Chromosome Disorders / Q000235:genetics; D002897:Chromosomes, Human, Pair 7 / Q000235:genetics; D003561:Cytarabine / Q000008:administration & dosage; D004252:DNA Mutational Analysis; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D016162:Genes, Wilms Tumor; D005819:Genetic Markers / Q000235:genetics; D006801:Humans; D015255:Idarubicin / Q000008:administration & dosage; D007223:Infant; D053208:Kaplan-Meier Estimate; D007621:Karyotyping; D015470:Leukemia, Myeloid, Acute / Q000188:drug therapy / Q000235:genetics* / Q000401:mortality; D008297:Male; D009190:Myelodysplastic Syndromes / Q000235:genetics; D009687:Nuclear Proteins / Q000235:genetics; D000090243:Nucleophosmin; D011379:Prognosis; D015996:Survival Rate; D014178:Translocation, Genetic / Q000235:genetics; D051941:fms-Like Tyrosine Kinase 3
chemical_list: D005819:Genetic Markers; C000716967:NPM1 protein, human; D009687:Nuclear Proteins; D003561:Cytarabine; D000090243:Nucleophosmin; D005047:Etoposide; C497970:FLT3 protein, human; D051941:fms-Like Tyrosine Kinase 3; D015255:Idarubicin