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dc.contributor.authorEspersen, Anne Dorte Lerche
dc.contributor.authorNoren-Nyström, Ulrika
dc.contributor.authorAbrahamsson, Jonas
dc.contributor.authorHa, Shau-Yin
dc.contributor.authorPronk, Cornelis Jan
dc.contributor.authorJahnukainen, Kirsi
dc.contributor.authorJónsson, Ólafur G.
dc.contributor.authorLausen, Birgitte
dc.contributor.authorPalle, Josefine
dc.contributor.authorZeller, Bernward
dc.contributor.authorPalmqvist, Lars
dc.contributor.authorHasle, Henrik
dc.date.accessioned2018-06-15T15:30:43Z
dc.date.available2018-06-15T15:30:43Z
dc.date.issued2018-07
dc.date.submitted2018
dc.identifier.citationAcute myeloid leukemia (AML) with t(7;12)(q36;p13) is associated with infancy and trisomy 19: Data from Nordic Society for Pediatric Hematology and Oncology (NOPHO-AML) and review of the literature 2018, 57 (7):359 Genes, Chromosomes and Canceren
dc.identifier.issn10452257
dc.identifier.doi10.1002/gcc.22538
dc.identifier.urihttp://hdl.handle.net/2336/620599
dc.descriptionTo access publisher's full text version of this article click on the hyperlink belowen
dc.description.abstractThe t(7;12)(q36;p13) (MNX1/ETV6) is not included in the WHO classification but has been described in up to 30% of acute myeloid leukemia (AML) in children <2 years and associated with a poor prognosis. We present the clinical and cytogenetics characteristics of AML cases with t(7;12)(p36;p13). A literature review identified 35 patients with this translocation, published between 2000 and 2015. Outcome data were available in 22 cases. The NOPHO-AML (Nordic Society for Pediatric Hematology and Oncology) database contained 651 patients with AML from 1993 to 2014 and seven (1.1%) had the translocation. The t(7;12) was only present in patients <2 years of age (median age 6 months) but none was diagnosed as newborn. These patients constituted 4.3% of the patients <2 years of age. There was a strong association with trisomy 19 (literature: 86%, NOPHO: 100%) and +8 (literature: 19%, NOPHO: 14%). Seventeen of 22 patients from the literature with t(7;12) and four of seven patients from the NOPHO database suffered from relapse. The patients with t(7;12) had a 3-year event free survival of 24% (literature) vs. 43% (NOPHO) and a 3-year overall survival of 42% (literature) vs. 100% (NOPHO). None of the NOPHO patients was treated with hematopoietic stem cell transplantation (HSCT) in first complete remission. Relapse was frequent but the salvage rate using HSCT was high. We conclude that t(7;12)(q36;13) is a unique subgroup of childhood AML with presentation before 2 years of age with most cases being associated with +19.
dc.description.sponsorshipDanish Childhood Cancer Foundationen
dc.language.isoenen
dc.publisherWileyen
dc.relation.urlhttp://doi.wiley.com/10.1002/gcc.22538en
dc.rightsArchived with thanks to Genes, Chromosomes and Canceren
dc.subjectBráðahvítblæðien
dc.subjectHvítblæðien
dc.subjectBörnen
dc.subjectGenen
dc.subjectPED12en
dc.subject.meshLeukemia, Myeloid, Acuteen
dc.subject.meshInfanten
dc.subject.meshCytogeneticsen
dc.subject.meshGenesen
dc.titleAcute myeloid leukemia (AML) with t(7;12)(q36;p13) is associated with infancy and trisomy 19: Data from Nordic Society for Pediatric Hematology and Oncology (NOPHO-AML) and review of the literatureen
dc.typeArticleen
dc.contributor.department[ 1 ] Aarhus Univ Hosp Skejby, Dept Pediat, Aarhus, Denmark Show more [ 2 ] Umea Univ Hosp, Dept Pediat, Umea, Sweden [ 3 ] Queen Silvia Childrens Hosp, Dept Pediat, Inst Clin Sci, Gothenburg, Sweden Show more [ 4 ] Queen Mary Hosp, Dept Pediat, Hong Kong, Hong Kong, Peoples R China [ 5 ] HKPHOSG, Hong Kong, Hong Kong, Peoples R China Show more [ 6 ] Univ Hosp, Dept Pediat, Lund, Sweden Show more [ 7 ] Univ Helsinki, Childrens Hosp, Helsinki, Finland Show more [ 8 ] Univ Helsinki, Cent Hosp, Helsinki, Finland [ 9 ] Landspitalinn, Dept Pediat, Reykjavik, Iceland Show more [ 10 ] Univ Copenhagen, Rigshosp, Dept Pediat & Adolescent Med, Copenhagen, Denmark Show more [ 11 ] Uppsala Univ, Dept Womans & Childrens Hlth, Uppsala, Sweden Show more [ 12 ] Oslo Univ Hosp, Div Pediat & Adolescent Med, Oslo, Norway Show more [ 13 ] Univ Gothenburg, Sahlgrenska Acad, Inst Biomed, Dept Clin Chem & Transfus Med, Gothenburg, Swedenen
dc.identifier.journalGenes, Chromosomes and Canceren
dc.rights.accessLandspitali Access - LSH-aðganguren
dc.contributor.institutionDepartment of Pediatrics; Aarhus University Hospital Skejby; Denmark
dc.contributor.institutionDepartment of Pediatrics; Umeå University Hospital; Umeå Sweden
dc.contributor.institutionInstitution for Clinical Sciences, Department of Pediatrics; Queen Silvia Children's Hospital; Gothenburg Sweden
dc.contributor.institutionDepartment of Pediatrics; Queen Mary Hospital and Hong Kong Pediatric Hematology & Oncology Study Group (HKPHOSG); Hong Kong China
dc.contributor.institutionDepartment of Pediatrics; University Hospital; Lund Sweden
dc.contributor.institutionChildren's Hospital, University of Helsinki and Helsinki University Central Hospital; Helsinki Finland
dc.contributor.institutionDepartment of Pediatrics; Landspitalinn; Reykjavik Iceland
dc.contributor.institutionDepartment of Pediatrics and Adolescent Medicine; Rigshospitalet, University of Copenhagen; Copenhagen Denmark
dc.contributor.institutionDepartment of Woman's and Children's Health; Uppsala University; Uppsala Sweden
dc.contributor.institutionDivision of Pediatric and Adolescent Medicine; Oslo University Hospital; Oslo Norway
dc.contributor.institutionDepartment of Clinical Chemistry and Transfusion Medicine; Institute of Biomedicine, Sahlgrenska Academy at University of Gothenburg; Gothenburg Sweden
dc.contributor.institutionDepartment of Pediatrics; Aarhus University Hospital Skejby; Denmark
dc.departmentcodePED12
html.description.abstractThe t(7;12)(q36;p13) (MNX1/ETV6) is not included in the WHO classification but has been described in up to 30% of acute myeloid leukemia (AML) in children <2 years and associated with a poor prognosis. We present the clinical and cytogenetics characteristics of AML cases with t(7;12)(p36;p13). A literature review identified 35 patients with this translocation, published between 2000 and 2015. Outcome data were available in 22 cases. The NOPHO-AML (Nordic Society for Pediatric Hematology and Oncology) database contained 651 patients with AML from 1993 to 2014 and seven (1.1%) had the translocation. The t(7;12) was only present in patients <2 years of age (median age 6 months) but none was diagnosed as newborn. These patients constituted 4.3% of the patients <2 years of age. There was a strong association with trisomy 19 (literature: 86%, NOPHO: 100%) and +8 (literature: 19%, NOPHO: 14%). Seventeen of 22 patients from the literature with t(7;12) and four of seven patients from the NOPHO database suffered from relapse. The patients with t(7;12) had a 3-year event free survival of 24% (literature) vs. 43% (NOPHO) and a 3-year overall survival of 42% (literature) vs. 100% (NOPHO). None of the NOPHO patients was treated with hematopoietic stem cell transplantation (HSCT) in first complete remission. Relapse was frequent but the salvage rate using HSCT was high. We conclude that t(7;12)(q36;13) is a unique subgroup of childhood AML with presentation before 2 years of age with most cases being associated with +19.


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