title: Increased risk for CNS relapse in pre-B cell leukemia with the t(1;19)/TCF3-PBX1.
abstract: To evaluate the impact of contemporary therapy on the clinical outcome of children with pre-B acute lymphoblastic leukemia (ALL) and the t(1;19)/TCF3/PBX1, we analyzed 735 patients with B-cell precursor ALL treated in four successive protocols at St Jude Children's Research Hospital. The 41 patients with the t(1;19) had a comparable event-free survival to that of the 694 patients with other B-cell precursor ALL (P=0.63; 84.2+/-7.1% (s.e.) vs 84.0+/-1.8% at 5 years). However, patients with the t(1;19) had a lower cumulative incidence of any hematological relapse (P=0.06; 0 vs 8.3+/-1.2% at 5 years) but a significantly higher incidence of central nervous system (CNS) relapse (P<0.001; 9.0+/-5.1% vs 1.0+/-0.4% at 5 years). In a multivariate analysis, the t(1;19) was an independent risk factor for isolated CNS relapse. These data suggest that with contemporary treatment, patients with the t(1;19) and TCF3/PBX1 fusion have a favorable overall outcome but increased risk of CNS relapse.
authors.affiliation: Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
authors.forename: S
authors.initials: S
authors.lastname: Jeha
authors.forename: D
authors.initials: D
authors.lastname: Pei
authors.forename: S C
authors.initials: SC
authors.lastname: Raimondi
authors.forename: M
authors.initials: M
authors.lastname: Onciu
authors.forename: D
authors.initials: D
authors.lastname: Campana
authors.forename: C
authors.initials: C
authors.lastname: Cheng
authors.forename: J T
authors.initials: JT
authors.lastname: Sandlund
authors.forename: R C
authors.initials: RC
authors.lastname: Ribeiro
authors.forename: J E
authors.initials: JE
authors.lastname: Rubnitz
authors.forename: S C
authors.initials: SC
authors.lastname: Howard
authors.forename: J R
authors.initials: JR
authors.lastname: Downing
authors.forename: W E
authors.initials: WE
authors.lastname: Evans
authors.forename: M V
authors.initials: MV
authors.lastname: Relling
authors.forename: C-H
authors.initials: CH
authors.lastname: Pui
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000008:administration & dosage / Q000627:therapeutic use; D002490:Central Nervous System / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics / Q000648:ultrastructure*; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics / Q000648:ultrastructure*; D002986:Clinical Trials as Topic / Q000706:statistics & numerical data; D003131:Combined Modality Therapy; D016371:Cranial Irradiation; D018572:Disease-Free Survival; D005260:Female; D005838:Genotype; D006801:Humans; D015994:Incidence; D007223:Infant; D007278:Injections, Spinal; D017254:Leukemic Infiltration / Q000453:epidemiology* / Q000517:prevention & control; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics*; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000453:epidemiology / Q000235:genetics* / Q000473:pathology / Q000532:radiotherapy; D011379:Prognosis; D016016:Proportional Hazards Models; D012306:Risk; D018570:Risk Assessment; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D015514:Oncogene Proteins, Fusion; C543638:TCF3-PBX1 fusion protein, human