title: Prognostic impact of t(1;19)/ TCF3-PBX1 in childhood acute lymphoblastic leukemia in the context of Berlin-Frankfurt-Münster-based protocols.
abstract: Historically, t(1;19)(q23;p13.3) has been related to pre-B acute lymphoblastic leukemia (ALL) and associated with a poor prognosis. Current treatments have overcome this dismal outcome, but advantages in survival for the unbalanced group have been reported. We compared the outcome of balanced and unbalanced der(19)t(1;19) cases and also patients with t(1;19)/TCF3-PBX1 versus patients without this translocation, to assess its prognostic value. From January 1990 to December 2010, t(1;19)(q23;p13)/TCF3-PBX1 was detected in 48 cases. Patients were treated with Berlin-Frankfurt-Münster (BFM)-based protocols and classified into balanced (n = 17) and unbalanced (n = 23) groups. The probability of event-free survival (pEFS) (standard error) of patients with t(1;19)/TCF3-PBX1 was 85% (6%), for the unbalanced group 78% (10%), and 88% (8%) for the balanced. The pEFS of patients with t(1;19)/TCF3-PBX1 was significantly superior to that of patients without t(1;19)/TCF3-PBX1 (p-value <0.0001). Patients with t(1;19)/TCF3-PBX1 presented a good outcome with no differences between balanced and unbalanced subgroups. Thus, risk-adjustment therapy would not be necessary for cases with t(1;19)/TCF3-PBX1.
authors.affiliation: Department of Hematology/Oncology, Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. marisa_felice@yahoo.com.ar
authors.forename: María S
authors.initials: MS
authors.lastname: Felice
authors.forename: Marta S
authors.initials: MS
authors.lastname: Gallego
authors.forename: Cristina N
authors.initials: CN
authors.lastname: Alonso
authors.forename: Elizabeth M
authors.initials: EM
authors.lastname: Alfaro
authors.forename: Myriam R
authors.initials: MR
authors.lastname: Guitter
authors.forename: Andrea R
authors.initials: AR
authors.lastname: Bernasconi
authors.forename: Patricia L
authors.initials: PL
authors.lastname: Rubio
authors.forename: Pedro A
authors.initials: PA
authors.lastname: Zubizarreta
authors.forename: Jorge G
authors.initials: JG
authors.lastname: Rossi
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001215:Asparaginase / Q000627:therapeutic use; D051792:Basic Helix-Loop-Helix Proteins / Q000235:genetics*; D002648:Child; D002675:Child, Preschool; D020732:Cytogenetic Analysis; D004268:DNA-Binding Proteins / Q000235:genetics*; D003630:Daunorubicin / Q000627:therapeutic use; D018572:Disease-Free Survival; D006801:Humans; D016130:Immunophenotyping; D007223:Infant; D000075062:Pre-B-Cell Leukemia Transcription Factor 1; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000175:diagnosis / Q000188:drug therapy / Q000235:genetics; D011241:Prednisone / Q000627:therapeutic use; D011379:Prognosis; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D014178:Translocation, Genetic / Q000235:genetics*; 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; C118089:TFPT protein, human; C083400:PBX1 protein, human