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

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Hdl Handle:
http://hdl.handle.net/2336/66773
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
Authors:
Forestier, Erik; Gauffin, Fredrika; Andersen, Mette K; Autio, Kirsi; Borgström, Georg; Golovleva, Irina; Gustafsson, Britt; Heim, Sverre; Heinonen, Kristina; Heyman, Mats; Hovland, Randi; Johannsson, Johann H; Kerndrup, Gitte; Rosenquist, Richard; Schoumans, Jacqueline; Swolin, Birgitta; Johansson, Bertil; Nordgren, Ann
Citation:
Genes Chromosomes Cancer. 2008, 47(2):149-58
Issue Date:
1-Feb-2008
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.
Description:
To access publisher full text version of this article. Please click on the hyperlink in Additional Links field
Additional Links:
http://dx.doi.org/10.1002/gcc.20517

Full metadata record

DC FieldValue Language
dc.contributor.authorForestier, Erik-
dc.contributor.authorGauffin, Fredrika-
dc.contributor.authorAndersen, Mette K-
dc.contributor.authorAutio, Kirsi-
dc.contributor.authorBorgström, Georg-
dc.contributor.authorGolovleva, Irina-
dc.contributor.authorGustafsson, Britt-
dc.contributor.authorHeim, Sverre-
dc.contributor.authorHeinonen, Kristina-
dc.contributor.authorHeyman, Mats-
dc.contributor.authorHovland, Randi-
dc.contributor.authorJohannsson, Johann H-
dc.contributor.authorKerndrup, Gitte-
dc.contributor.authorRosenquist, Richard-
dc.contributor.authorSchoumans, Jacqueline-
dc.contributor.authorSwolin, Birgitta-
dc.contributor.authorJohansson, Bertil-
dc.contributor.authorNordgren, Ann-
dc.date.accessioned2009-04-30T14:26:33Z-
dc.date.available2009-04-30T14:26:33Z-
dc.date.issued2008-02-01-
dc.date.submitted2009-04-30-
dc.identifier.citationGenes Chromosomes Cancer. 2008, 47(2):149-58en
dc.identifier.issn1098-2264-
dc.identifier.pmid17990329-
dc.identifier.doi10.1002/gcc.20517-
dc.identifier.urihttp://hdl.handle.net/2336/66773-
dc.descriptionTo access publisher full text version of this article. Please click on the hyperlink in Additional Links fielden
dc.description.abstractAlthough 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.en
dc.language.isoenen
dc.publisherWiley-Lissen
dc.relation.urlhttp://dx.doi.org/10.1002/gcc.20517en
dc.subject.meshAdolescenten
dc.titleClinical 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 literatureen
dc.typeArticleen
dc.contributor.departmentDepartment of Clinical Sciences, Pediatrics, University of Umeå, Umeå, Sweden. ann.nordgren@ki.seen
dc.identifier.journalGenes, chromosomes & canceren

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