Increased risk for CNS relapse in pre-B cell leukemia with the t(1;19)/TCF3-PBX1.
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.
Jeha
S
S
Department of Oncology, St Jude Children's Research Hospital, Memphis, TN 38105, USA.
Pei
D
D
Raimondi
S C
SC
Onciu
M
M
Campana
D
D
Cheng
C
C
Sandlund
J T
JT
Ribeiro
R C
RC
Rubnitz
J E
JE
Howard
S C
SC
Downing
J R
JR
Evans
W E
WE
Relling
M V
MV
Pui
C-H
CH
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
D015514:Oncogene Proteins, Fusion; C543638:TCF3-PBX1 fusion protein, human