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.

Details

Serval ID
serval:BIB_CD26B9D0E6A6
Type
Article: article from journal or magazin.
Publication sub-type
Case report (case report): feedback on an observation with a short commentary.
Collection
Publications
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.
Journal
Genes, Chromosomes and Cancer
Author(s)
Forestier E., Gauffin F., Andersen M.K., Autio K., Borgström G., Golovleva I., Gustafsson B., Heim S., Heinonen K., Heyman M., Hovland R., Johannsson J.H., Kerndrup G., Rosenquist R., Schoumans J., Swolin B., Johansson B., Nordgren A., Oncology 
Working group(s)
Nordic Society of Pediatric Hematology, Swedish Cytogenetic Leukemia Study Group, NOPHO Leukemia Cytogenetic Study Group
ISSN
1098-2264 (Electronic)
ISSN-L
1045-2257
Publication state
Published
Issued date
2008
Volume
47
Number
2
Pages
149-158
Language
english
Notes
Publication types: Case Reports ; Journal Article ; Research Support, Non-U.S. Gov't ; ReviewPublication Status: ppublish
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, CD10, CD19, 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)/l, 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.
Keywords
Adolescent, Child, Child, Preschool, Chromosomes, Human, Pair 20/genetics, Chromosomes, Human, Pair 9/genetics, Cytogenetics, Female, Humans, Infant, Infant, Newborn, Karyotyping, Leukemia, B-Cell/genetics, Male, Precursor Cell Lymphoblastic Leukemia-Lymphoma/genetics, Type="Geographic">Scandinavia, Translocation, Genetic
Pubmed
Web of science
Create date
17/09/2011 10:06
Last modification date
20/08/2019 16:47
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