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Clinical and cytoge...
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.
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- Karrman, Kristina (author)
- Lund University,Lunds universitet,Avdelningen för klinisk genetik,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Clinical Genetics,Department of Laboratory Medicine,Faculty of Medicine,Department of Clinical Genetics, Lund University Hospital, Lund, Sweden,Department of Clinical Genetics, Rigshospitalet, Copenhagen, Denmark
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- Forestier, Erik (author)
- Umeå universitet,Pediatrik
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- Heyman, Mats (author)
- Karolinska Institutet
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- Andersen, Mette K (author)
- Department of Pathology, Odense University Hospital, Odense, Denmark
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- Autio, Kirsi (author)
- Department of Pathology and Clinical Genetics, Haartman Institute, University of Helsinki, Helsinki, Finland
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- Blennow, Elisabeth (author)
- Karolinska Institutet
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- Borgström, Georg (author)
- Department of Pathology and Clinical Genetics, Haartman Institute, University of Helsinki, Helsinki, Finland
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- Ehrencrona, Hans (author)
- Uppsala universitet,Uppsala University,Department of Genetics and Pathology, Uppsala University, Uppsala, Sweden,Institutionen för genetik och patologi
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- Golovleva, Irina (author)
- Umeå universitet,Medicinsk och klinisk genetik
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- Heim, Sverre (author)
- Faculty of Medicine, University of Oslo, Oslo, Norway
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- Heinonen, Kristiina (author)
- Genetic Laboratory, ISLAB, Kuopio, Finland
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- Hovland, Randi (author)
- Center of Medical Genetics and Molecular Medicine, Haukeland University Hospital, Helse-Bergen HF, Norway
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- Johannsson, Johann H (author)
- Department of Clinical Genetics and Cytogenetics, University Hospital, Reykjavik, Iceland
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Kerndrup, Gitte (author)
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- Nordgren, Ann (author)
- Karolinska Institutet
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- Palmqvist, Lars, 1965 (author)
- Gothenburg University,Göteborgs universitet,Institutionen för biomedicin, avdelningen för klinisk kemi och transfusionsmedicin,Institute of Biomedicine, Department of Clinical Chemistry and Transfusion Medicine,Department of Clinical Chemistry and Transfusion Medicine, Sahlgrenska University Hospital, Göteborg, Sweden
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- Johansson, Bertil (author)
- Lund University,Lunds universitet,Avdelningen för klinisk genetik,Institutionen för laboratoriemedicin,Medicinska fakulteten,Division of Clinical Genetics,Department of Laboratory Medicine,Faculty of Medicine,Department of Clinical Genetics, Lund University Hospital, Lund, Sweden
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(creator_code:org_t)
- Wiley, 2009
- 2009
- English.
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In: Genes, chromosomes & cancer. - : Wiley. - 1098-2264 .- 1045-2257. ; 48:9, s. 795-805
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Abstract
Subject headings
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- 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.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Medicinsk genetik (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Medical Genetics (hsv//eng)
Keyword
- 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
- diagnosis
- genetics
- Prognosis
- Proportional Hazards Models
- Receptors
- Antigen
- T-Cell
- genetics
- Kaplan-Meier Estimate
- MEDICINE
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Karrman, Kristin ...
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Forestier, Erik
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Heyman, Mats
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Andersen, Mette ...
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Autio, Kirsi
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Blennow, Elisabe ...
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show more...
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Borgström, Georg
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Ehrencrona, Hans
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Golovleva, Irina
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Heim, Sverre
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Heinonen, Kristi ...
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Hovland, Randi
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Johannsson, Joha ...
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Kerndrup, Gitte
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Nordgren, Ann
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Palmqvist, Lars, ...
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Johansson, Berti ...
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- About the subject
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Clinical Medicin ...
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and Hematology
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- MEDICAL AND HEALTH SCIENCES
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MEDICAL AND HEAL ...
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and Basic Medicine
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and Medical Genetics
- Articles in the publication
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Genes, chromosom ...
- By the university
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University of Gothenburg
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Lund University
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Umeå University
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Karolinska Institutet
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Uppsala University