title: Incidence and outcome of TCF3-PBX1-positive acute lymphoblastic leukemia in Austrian children.
abstract: Lessons from the analysis of children with TCF3-PBX1 ALL could help to identify treatment components essential for this leukemia subtype. Of 859 children with ALL who were treated in ALL-BFM trials in Austria, 31 (3.6%) had a TCF3-PBX1 ALL. The 5-year event-free survival rate for these 31 patients was 90%+/-5%. Patients with TCF3-PBX1 ALL treated on the ALL-BFM 86 trial had a poorer outcome than patients with TCF3-PBX1 ALL treated on later trials. These data document that contemporary ALL-BFM treatment is highly effective in children with TCF3-PBX1 ALL. Implementation of early dose-intensified remission induction may be an essential treatment component.
authors.affiliation: Department of Hematology and Oncology, St. Anna Children's Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria.
authors.forename: Leo
authors.initials: L
authors.lastname: Kager
authors.forename: Thomas
authors.initials: T
authors.lastname: Lion
authors.forename: Andishe
authors.initials: A
authors.lastname: Attarbaschi
authors.forename: Margit
authors.initials: M
authors.lastname: Koenig
authors.forename: Sabine
authors.initials: S
authors.lastname: Strehl
authors.forename: Oskar A
authors.initials: OA
authors.lastname: Haas
authors.forename: Michael N
authors.initials: MN
authors.lastname: Dworzak
authors.forename: Martin
authors.initials: M
authors.lastname: Schrappe
authors.forename: Helmut
authors.initials: H
authors.lastname: Gadner
authors.forename: Georg
authors.initials: G
authors.lastname: Mann
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001215:Asparaginase / Q000627:therapeutic use; D001317:Austria / Q000453:epidemiology; D051792:Basic Helix-Loop-Helix Proteins / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D004268:DNA-Binding Proteins / Q000235:genetics; D003630:Daunorubicin / Q000627:therapeutic use; D006801:Humans; D015994:Incidence; D007223:Infant; D000075062:Pre-B-Cell Leukemia Transcription Factor 1; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy* / Q000453:epidemiology* / Q000401:mortality; D011241:Prednisone / Q000627:therapeutic use; D011518:Proto-Oncogene Proteins / Q000235:genetics; D015996:Survival Rate; D016896:Treatment Outcome; D014750:Vincristine / Q000627:therapeutic use
chemical_list: D001215:Asparaginase; D051792:Basic Helix-Loop-Helix Proteins; D004268:DNA-Binding Proteins; D003630:Daunorubicin; D000075062:Pre-B-Cell Leukemia Transcription Factor 1; D011241:Prednisone; D011518:Proto-Oncogene Proteins; D014750:Vincristine; C407909:TCF3 protein, human; C083400:PBX1 protein, human