Abstract
| Originalsprog | Engelsk |
|---|---|
| Tidsskrift | Genes, Chromosomes & Cancer |
| Vol/bind | 48 |
| Udgave nummer | 9 |
| Sider (fra-til) | 795-805 |
| Antal sider | 10 |
| ISSN | 1045-2257 |
| DOI | |
| Status | Udgivet - 2009 |
Bibliografisk note
Keywords: Adolescent; Chi-Square Distribution; Child; Child, Preschool; Chromosome Aberrations; Cohort Studies; Cytogenetic Analysis; Female; Gene Rearrangement, T-Lymphocyte; Humans; Kaplan-Meiers Estimate; Male; Multivariate Analysis; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma; Prognosis; Proportional Hazards Models; Receptors, Antigen, T-CellCitationsformater
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I: Genes, Chromosomes & Cancer, Bind 48, Nr. 9, 2009, s. 795-805.
Publikation: Bidrag til tidsskrift › Tidsskriftartikel › Forskning › peer review
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TY - JOUR
T1 - Clinical and cytogenetic features of a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias: rare T-cell receptor gene rearrangements are associated with poor outcome
AU - Karrman, Kristina
AU - Forestier, Erik
AU - Heyman, Mats
AU - Andersen, Mette K
AU - Autio, Kirsi
AU - Blennow, Elisabeth
AU - Borgström, Georg
AU - Ehrencrona, Hans
AU - Golovleva, Irina
AU - Heim, Sverre
AU - Heinonen, Kristiina
AU - Hovland, Randi
AU - Johannsson, Johann H
AU - Kerndrup, Gitte
AU - Nordgren, Ann
AU - Palmqvist, Lars
AU - Johansson, Bertil
AU - Nordic Society of Pediatric Hematology, Oncology (NOPHO)
AU - Swedish Cytogenetic Leukemia Study Group (SCLSG)
AU - NOPHO Leukemia Cytogenetic Study Group (NLCSG)
N1 - Keywords: Adolescent; Chi-Square Distribution; Child; Child, Preschool; Chromosome Aberrations; Cohort Studies; Cytogenetic Analysis; Female; Gene Rearrangement, T-Lymphocyte; Humans; Kaplan-Meiers Estimate; Male; Multivariate Analysis; Precursor T-Cell Lymphoblastic Leukemia-Lymphoma; Prognosis; Proportional Hazards Models; Receptors, Antigen, T-Cell
PY - 2009
Y1 - 2009
N2 - Clinical characteristics and cytogenetic aberrations were ascertained and reviewed in a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias (T-ALLs) diagnosed between 1992 and 2006 in the Nordic countries. Informative karyotypic results were obtained in 249 (87%) cases, of which 119 (48%) were cytogenetically abnormal. Most (62%) of the aberrant T-ALLs were pseudodiploid. Structural changes were more common than numerical ones; 86% displayed at least one structural abnormality and 41% at least one numerical anomaly. The most frequent abnormalities were T-cell receptor (TCR) gene rearrangements (20%) [TCR;11p13 (10%), TCR;10q24 (3%), TCR;other (8%)], del(9p) (17%), +8 (14%), del(6q) (12%), and 11q23 rearrangements (6%). The TCR;other group comprised the rare rearrangements t(X;14)(p11;q11), t(X;7)(q22;q34), t(1;14)(p32;q11), ins(14;5)(q11;q?q?), inv(7)(p15q34), t(8;14)(q24;q11), t(7;11)(q34;p15), and t(12;14)(p13;q11). The clinical characteristics of this Nordic patient cohort agreed well with previous larger series, with a median age of 9.0 years, male predominance (male/female ratio 3.1), median white blood cell (WBC) count of 66.5 x 10(9)/l, and a high incidence of mediastinal mass and central nervous system involvement (59% and 9.5%, respectively). These features did not differ significantly among the various genetic subgroups. 5-year event-free survival (EFS) and overall survival for all patients were 0.61 (+/-0.03) and 0.67 (+/-0.03), respectively. In a multivariate analysis, two factors affected negatively the EFS, namely a WBC count of > or =200 x 10(9)/l (P < 0.001) and the presence of rare TCR rearrangements (P = 0.001). In conclusion, in this large series of childhood T-ALLs from the Nordic countries, the cytogenetic findings were not associated with risk of therapy failure with the exception of the TCR;other group. However, further prospective and collaborative investigations of this genetically heterogeneous entity are needed to confirm these results.
AB - Clinical characteristics and cytogenetic aberrations were ascertained and reviewed in a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias (T-ALLs) diagnosed between 1992 and 2006 in the Nordic countries. Informative karyotypic results were obtained in 249 (87%) cases, of which 119 (48%) were cytogenetically abnormal. Most (62%) of the aberrant T-ALLs were pseudodiploid. Structural changes were more common than numerical ones; 86% displayed at least one structural abnormality and 41% at least one numerical anomaly. The most frequent abnormalities were T-cell receptor (TCR) gene rearrangements (20%) [TCR;11p13 (10%), TCR;10q24 (3%), TCR;other (8%)], del(9p) (17%), +8 (14%), del(6q) (12%), and 11q23 rearrangements (6%). The TCR;other group comprised the rare rearrangements t(X;14)(p11;q11), t(X;7)(q22;q34), t(1;14)(p32;q11), ins(14;5)(q11;q?q?), inv(7)(p15q34), t(8;14)(q24;q11), t(7;11)(q34;p15), and t(12;14)(p13;q11). The clinical characteristics of this Nordic patient cohort agreed well with previous larger series, with a median age of 9.0 years, male predominance (male/female ratio 3.1), median white blood cell (WBC) count of 66.5 x 10(9)/l, and a high incidence of mediastinal mass and central nervous system involvement (59% and 9.5%, respectively). These features did not differ significantly among the various genetic subgroups. 5-year event-free survival (EFS) and overall survival for all patients were 0.61 (+/-0.03) and 0.67 (+/-0.03), respectively. In a multivariate analysis, two factors affected negatively the EFS, namely a WBC count of > or =200 x 10(9)/l (P < 0.001) and the presence of rare TCR rearrangements (P = 0.001). In conclusion, in this large series of childhood T-ALLs from the Nordic countries, the cytogenetic findings were not associated with risk of therapy failure with the exception of the TCR;other group. However, further prospective and collaborative investigations of this genetically heterogeneous entity are needed to confirm these results.
U2 - 10.1002/gcc.20684
DO - 10.1002/gcc.20684
M3 - Journal article
C2 - 19530250
SN - 1045-2257
VL - 48
SP - 795
EP - 805
JO - Genes, Chromosomes & Cancer
JF - Genes, Chromosomes & Cancer
IS - 9
ER -