Incidence and outcome of TCF3-PBX1-positive acute lymphoblastic leukemia in Austrian children.
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.
Kager
Leo
L
Department of Hematology and Oncology, St. Anna Children's Hospital, Kinderspitalgasse 6, A-1090 Vienna, Austria.
Lion
Thomas
T
Attarbaschi
Andishe
A
Koenig
Margit
M
Strehl
Sabine
S
Haas
Oskar A
OA
Dworzak
Michael N
MN
Schrappe
Martin
M
Gadner
Helmut
H
Mann
Georg
G
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
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