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Clinical and cytogenetic features of pediatric dic(9;20)(p13.2;q11.2)-positive B-Cell precursor acute lymphoblastic leukemias: A nordic series of 24 cases and review of the literature

Forestier, Erik; Gauffin, Fredrika; Andersen, Mette K.; Autio, Kirsi; Borgstrom, Georg; Golovleva, Irina; Gustafsson, Britt; Heim, Sverre; Heinonen, Kristina and Heyman, Mats, et al. (2008) In Genes, Chromosomes and Cancer 47(2). p.149-158
Abstract
Although dic(9;20)(p13.2;q11.2) is a characteristic abnormality in childhood B-cell precursor acute lymphoblastic leukemias (BCP ALL), little is known about its clinical impact or the type and frequency of additional aberrations it may occur together with. We here review the clinical and cytogenetic features of a Nordic pediatric series of 24 patients with dic(9;20)-positive BCP ALL diagnosed 1996-2006, constituting 1.3% of the BCP ALL, as well as 47 childhood cases from the literature. Consistent immunophenotypic features of the Nordic cases included positivity for HLA-DR, CID 10, CID19, CD20, and CD22 and negativity for T-cell and myeloid markers; no detailed immunophenotypes were reported for the previously published cases. In the... (More)
Although dic(9;20)(p13.2;q11.2) is a characteristic abnormality in childhood B-cell precursor acute lymphoblastic leukemias (BCP ALL), little is known about its clinical impact or the type and frequency of additional aberrations it may occur together with. We here review the clinical and cytogenetic features of a Nordic pediatric series of 24 patients with dic(9;20)-positive BCP ALL diagnosed 1996-2006, constituting 1.3% of the BCP ALL, as well as 47 childhood cases from the literature. Consistent immunophenotypic features of the Nordic cases included positivity for HLA-DR, CID 10, CID19, CD20, and CD22 and negativity for T-cell and myeloid markers; no detailed immunophenotypes were reported for the previously published cases. In the entire cohort of 71 cases, the modal chromosome distribution was 45 (62%), 46 (21%), 47 (7%), 48 (4%), 49 (3%), 44 (1%), and 50 (1%). Additional changes were present in 63%, the most frequent of which were homozygous loss of CDKN2A (33%) and gains of chromosomes 21 (28%) and x (10%). The median patient age was 3 years, the female/male ratio was 2.0, the median white blood cell count was 24 x 10(9)/1, 11% had central nervous system involvement, and 5% had a mediastinal mass at diagnosis. Risk group stratification was nonstandard risk in 79%. The event-free survival and overall survival at 5 years for the 24 Nordic cases was 0.62 and 0.82, respectively. Thus, although relapses are quite common, postrelapse treatment of many patients is successful. This article contains Supplementary Material available at http://www.interscience.wiley.com/ jpages/1045-2257/suppmat. (Less)
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Genes, Chromosomes and Cancer
volume
47
issue
2
pages
149 - 158
publisher
John Wiley & Sons
external identifiers
  • wos:000252270900006
  • scopus:38049063130
ISSN
1045-2257
DOI
10.1002/gcc.20517
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English
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yes
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043e03fc-ba74-41d5-8e6f-49179c7ac68d (old id 1200070)
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2008-09-12 09:31:39
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2017-04-09 03:28:01
@article{043e03fc-ba74-41d5-8e6f-49179c7ac68d,
  abstract     = {Although dic(9;20)(p13.2;q11.2) is a characteristic abnormality in childhood B-cell precursor acute lymphoblastic leukemias (BCP ALL), little is known about its clinical impact or the type and frequency of additional aberrations it may occur together with. We here review the clinical and cytogenetic features of a Nordic pediatric series of 24 patients with dic(9;20)-positive BCP ALL diagnosed 1996-2006, constituting 1.3% of the BCP ALL, as well as 47 childhood cases from the literature. Consistent immunophenotypic features of the Nordic cases included positivity for HLA-DR, CID 10, CID19, CD20, and CD22 and negativity for T-cell and myeloid markers; no detailed immunophenotypes were reported for the previously published cases. In the entire cohort of 71 cases, the modal chromosome distribution was 45 (62%), 46 (21%), 47 (7%), 48 (4%), 49 (3%), 44 (1%), and 50 (1%). Additional changes were present in 63%, the most frequent of which were homozygous loss of CDKN2A (33%) and gains of chromosomes 21 (28%) and x (10%). The median patient age was 3 years, the female/male ratio was 2.0, the median white blood cell count was 24 x 10(9)/1, 11% had central nervous system involvement, and 5% had a mediastinal mass at diagnosis. Risk group stratification was nonstandard risk in 79%. The event-free survival and overall survival at 5 years for the 24 Nordic cases was 0.62 and 0.82, respectively. Thus, although relapses are quite common, postrelapse treatment of many patients is successful. This article contains Supplementary Material available at http://www.interscience.wiley.com/ jpages/1045-2257/suppmat.},
  author       = {Forestier, Erik and Gauffin, Fredrika and Andersen, Mette K. and Autio, Kirsi and Borgstrom, Georg and Golovleva, Irina and Gustafsson, Britt and Heim, Sverre and Heinonen, Kristina and Heyman, Mats and Hovland, Randi and Johannsson, Johann H. and Kerndrup, Gitte and Rosenquist, Richard and Schoumans, Jacqueline and Swolin, Birgitta and Johansson, Bertil and Nordgren, Ann},
  issn         = {1045-2257},
  language     = {eng},
  number       = {2},
  pages        = {149--158},
  publisher    = {John Wiley & Sons},
  series       = {Genes, Chromosomes and Cancer},
  title        = {Clinical and cytogenetic features of pediatric dic(9;20)(p13.2;q11.2)-positive B-Cell precursor acute lymphoblastic leukemias: A nordic series of 24 cases and review of the literature},
  url          = {http://dx.doi.org/10.1002/gcc.20517},
  volume       = {47},
  year         = {2008},
}