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

• Published in: British journal of haematology 2011-10, Vol.155 (2), p.235-243
• Main Authors: Andersen, Mette K., Autio, Kirsi, Barbany, Gisela, Borgström, Georg, Cavelier, Lucia, Golovleva, Irina, Heim, Sverre, Heinonen, Kristina, Hovland, Randi, Johannsson, Johann H., Johansson, Bertil, Kjeldsen, Eigil, Nordgren, Ann, Palmqvist, Lars, Forestier, Erik
• Format: Article
• Language: English
• Subjects: 19; Acute lymphatic leukemia; acute lymphoblastic leukaemia; Adolescence; Adolescent; Age; Aneuploidy; Antineoplastic Combined Chemotherapy Protocols - therapeutic use; Biological and medical sciences; Bone marrow; Central nervous system; Child; Child, Preschool; Children; Chromosome aberrations; Chromosome translocations; Chromosomes, Human, Pair 1 - genetics; Chromosomes, Human, Pair 1 - ultrastructure; Chromosomes, Human, Pair 12 - genetics; Chromosomes, Human, Pair 12 - ultrastructure; Chromosomes, Human, Pair 19 - genetics; Chromosomes, Human, Pair 19 - ultrastructure; Chromosomes, Human, Pair 21 - genetics; Chromosomes, Human, Pair 21 - ultrastructure; Clinical Medicine; Disease-Free Survival; Finland - epidemiology; Follow-Up Studies; Hematologi; Hematologic and hematopoietic diseases; Hematology; Humans; Iceland - epidemiology; Infant; Kaplan-Meier Estimate; Klinisk medicin; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Leukocytes; Lymphocytes B; Medical and Health Sciences; Medical Genetics; Medical sciences; Medicin och hälsovetenskap; Medicinsk genetik; Oncogene Proteins, Fusion - genetics; Oncology; paediatric; PBX1; Pediatrics; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - drug therapy; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - epidemiology; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - genetics; Precursor B-Cell Lymphoblastic Leukemia-Lymphoma - pathology; Prognosis; Recurrence; Scandinavian and Nordic Countries - epidemiology; Survival; t(1; 19); TCF3; Translocation, Genetic; Treatment Outcome
• ISSN: 0007-1048; 1365-2141; 1365-2141
• DOI: 10.1111/j.1365-2141.2011.08824.x

Summary 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 × 109/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).