title: Paediatric B-cell precursor acute lymphoblastic leukaemia with t(1;19)(q23;p13): clinical and cytogenetic characteristics of 47 cases from the Nordic countries treated according to NOPHO protocols.
abstract: The translocation t(1;19)(q23;p13)/der(19)t(1;19) is a risk stratifying aberration in childhood B-cell precursor acute lymphoblastic leukaemia (BCP ALL) in the Nordic countries. We have identified 47 children/adolescents with t(1;19)/der(19)t(1;19)-positive BCP ALL treated on two successive Nordic Society of Paediatric Haematology and Oncology (NOPHO) protocols between 1992 and 2007 and have reviewed the clinical and cytogenetic characteristics of these cases, comprising 1·8% of all cases. The translocation was balanced in 15 cases (32%) and unbalanced in 29 cases (62%). The most common additional chromosome abnormalities were del(9p), i(9q), del(6q), and del(13q). The median age was 7 years, the median white blood cell (WBC) count was 16 × 10(9)/l, and the female/male ratio was 1·2. The predicted event-free survival (EFS) at 5 and 10 years was 0·79, whereas the predicted overall survival (OS) at 5 and 10 years was 0·85 and 0·82, respectively. Nine patients had a bone marrow relapse after a median of 23 months; no patient had a central nervous system relapse. Additional cytogenetic abnormalities, age, gender, WBC count or whether the t(1;19) was balanced or unbalanced did not influence EFS or OS. Compared to cases with t(12,21) and high hyperdiploidy, EFS was similar, but overall survival was worse in patients with t(1;19)/der(19)t(1;19) (P = 0·004).
authors: Department of Clinical Genetics, Rigshospitalet, Copenhagen, Denmark. mette.klarskov.andersen@rh.regionh.dk
authors: Mette K
authors: MK
authors: Andersen
authors: Kirsi
authors: K
authors: Autio
authors: Gisela
authors: G
authors: Barbany
authors: Georg
authors: G
authors: Borgström
authors: Lucia
authors: L
authors: Cavelier
authors: Irina
authors: I
authors: Golovleva
authors: Sverre
authors: S
authors: Heim
authors: Kristina
authors: K
authors: Heinonen
authors: Randi
authors: R
authors: Hovland
authors: Johann H
authors: JH
authors: Johannsson
authors: Bertil
authors: B
authors: Johansson
authors: Eigil
authors: E
authors: Kjeldsen
authors: Ann
authors: A
authors: Nordgren
authors: Lars
authors: L
authors: Palmqvist
authors: Erik
authors: E
authors: Forestier
mesh_terms: D000293:Adolescent; D000782:Aneuploidy; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D002648:Child; D002675:Child, Preschool; D002878:Chromosomes, Human, Pair 1 / Q000235:genetics* / Q000648:ultrastructure; D002881:Chromosomes, Human, Pair 12 / Q000235:genetics / Q000648:ultrastructure; D002888:Chromosomes, Human, Pair 19 / Q000235:genetics* / Q000648:ultrastructure; D002891:Chromosomes, Human, Pair 21 / Q000235:genetics / Q000648:ultrastructure; D018572:Disease-Free Survival; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D006801:Humans; D007055:Iceland / Q000453:epidemiology; D007223:Infant; D053208:Kaplan-Meier Estimate; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D015452:Precursor B-Cell Lymphoblastic Leukemia-Lymphoma / Q000188:drug therapy / Q000453:epidemiology* / Q000235:genetics / Q000473:pathology; D011379:Prognosis; D012008:Recurrence; D012537:Scandinavian and Nordic Countries / Q000453:epidemiology; D014178:Translocation, Genetic; D016896:Treatment Outcome
chemical_list: D015514:Oncogene Proteins, Fusion; C543638:TCF3-PBX1 fusion protein, human