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Clinical and geneti...
Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries
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- Lundin, Catarina (author)
- Lund University,Lunds universitet,Avdelningen för klinisk genetik,Institutionen för laboratoriemedicin,Medicinska fakulteten,Genetiska och epigenetiska studier av barnleukemi,Forskargrupper vid Lunds universitet,Division of Clinical Genetics,Department of Laboratory Medicine,Faculty of Medicine,Genetic and epigenetic studies of pediatric leukemia,Lund University Research Groups
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- Forestier, Erik (author)
- Umeå universitet,Medicinsk och klinisk genetik
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Andersen, Mette Klarskov (author)
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Autio, Kirsi (author)
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- Barbany, Gisela (author)
- Karolinska Institutet
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- Cavelier, Lucia (author)
- Uppsala universitet,Medicinsk genetik
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- Golovleva, Irina (author)
- Umeå universitet,Medicinsk och klinisk genetik
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Heim, Sverre (author)
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Heinonen, Kristiina (author)
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Hovland, Randi (author)
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Johannsson, Johann H. (author)
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- Kjeldsen, Eigil (author)
- Umeå universitet,Medicinsk och klinisk genetik
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- Nordgren, Ann (author)
- Karolinska Institutet
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Palmqvist, Lars (author)
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- Johansson, Bertil (author)
- Lund University,Lunds universitet,Avdelningen för klinisk genetik,Institutionen för laboratoriemedicin,Medicinska fakulteten,Genetiska och epigenetiska studier av barnleukemi,Forskargrupper vid Lunds universitet,Division of Clinical Genetics,Department of Laboratory Medicine,Faculty of Medicine,Genetic and epigenetic studies of pediatric leukemia,Lund University Research Groups
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(creator_code:org_t)
- 2014-04-11
- 2014
- English.
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In: Journal of Hematology & Oncology. - : Springer Science and Business Media LLC. - 1756-8722. ; 7, s. 32-
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Abstract
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- Background: Children with Down syndrome (DS) have an increased risk for acute lymphoblastic leukemia (ALL). Although previous studies have shown that DS-ALL differs clinically and genetically from non-DS-ALL, much remains to be elucidated as regards genetic and prognostic factors in DS-ALL. Methods: To address clinical and genetic differences between DS-ALL and non-DS-ALL and to identify prognostic factors in DS-ALL, we ascertained and reviewed all 128 pediatric DS-ALL diagnosed in the Nordic countries between 1981 and 2010. Their clinical and genetic features were compared with those of the 4,647 B-cell precursor (BCP) ALL cases diagnosed during the same time period. Results: All 128 DS-ALL were BCP ALL, comprising 2.7% of all such cases. The 5-year event-free survival (EFS) and overall survival (OS) were significantly (P = 0.026 and P = 0.003, respectively) worse for DS-ALL patients with white blood cell counts >= 50 x 10(9)/l. The age distributions varied between the DS and non-DS cases, with age peaks at 2 and 3 years, respectively; none of the DS patients had infant ALL (P = 0.029). The platelet counts were lower in the DS-ALL group (P = 0.005). Abnormal karyotypes were more common in non-DS-ALL (P < 0.0001), and there was a significant difference in the modal number distribution, with only 2% high hyperdiploid DS-ALL cases (P < 0.0001). The 5-year EFS and 5-year OS were significantly worse for DS-ALL (0.574 and 0.691, respectively) compared with non-DS-ALL (0.783 and 0.894, respectively) in the NOPHO ALL-1992/2000 protocols (P < 0.001). Conclusions: The present study adds further support for genetic and clinical differences between DS-ALL and non-DS-ALL.
Subject headings
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Hematologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Hematology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Klinisk medicin -- Cancer och onkologi (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Clinical Medicine -- Cancer and Oncology (hsv//eng)
- MEDICIN OCH HÄLSOVETENSKAP -- Medicinska och farmaceutiska grundvetenskaper -- Medicinsk genetik (hsv//swe)
- MEDICAL AND HEALTH SCIENCES -- Basic Medicine -- Medical Genetics (hsv//eng)
Keyword
- Down syndrome
- ALL
- Children
- Clinical features
- Genetic features
- Prognosis
- Down syndrome
- ALL
- Children
- Clinical features
- Genetic features
- Prognosis
Publication and Content Type
- ref (subject category)
- art (subject category)
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- By the author/editor
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Lundin, Catarina
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Forestier, Erik
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Andersen, Mette ...
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Autio, Kirsi
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Barbany, Gisela
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Cavelier, Lucia
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show more...
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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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Kjeldsen, Eigil
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Nordgren, Ann
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Palmqvist, Lars
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Johansson, Berti ...
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show less...
- 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 Clinical Medicin ...
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and Cancer and Oncol ...
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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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Journal of Hemat ...
- By the university
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Uppsala University
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Lund University
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Umeå University
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Karolinska Institutet