Show simple item record Blink M Zimmermann M Neuhoff CV Reinhardt D de Haas V Hasle H O'Brien MM Stark B Tandonnet J Pession A Tousovska K Cheuk DK Kudo K Taga T Rubnitz JE Haltrich, Irén Balwierz W Pieters R Forestier E Johansson B van den Heuvel-Eibrink MM Zwaan CM 2016-04-21T09:25:08Z 2016-04-21T09:25:08Z 2014
dc.identifier 84896716441
dc.identifier.citation pagination=299-307; journalVolume=99; journalIssueNumber=2; journalTitle=HAEMATOLOGICA: THE HEMATOLOGY JOURNAL;
dc.identifier.uri doi:10.3324/haematol.2013.089425
dc.description.abstract Myeloid leukemia of Down Syndrome has a better prognosis than sporadic pediatric acute myeloid leukemia. Most myeloid leukemia of Down syndrome cases are characterized by additional cytogenetic changes besides the constitutional trisomy 21, but their potential prognostic impact is not known. We therefore conducted an international retrospective study of clinical characteristics, cytogenetics, treatment, and outcome of 451 children with myeloid leukemia of Down syndrome . All karyotypes were centrally reviewed before assigning patients to subgroups. The overall 7-year event-free survival for the entire cohort was 78% (+/-2%), with overall survival 79% (+/-2%), cumulative incidence of relapse 12% (+/-2%), and cumulative incidence of toxic death 7% (+/-1%). Outcome estimates showed large differences across the different cytogenetic subgroups. Based on the cumulative incidence of relapse , we could risk-stratify patients into two groups: normal karyotype cases (n=103) with a higher cumulative incidence of relapse (21% (+/-4%)) than cases with an aberrant karyotype (n=255) with a cumulative incidence of relapse of 9% (+/-2%) (p=0.004). Multivariate analyses revealed white blood cell counts >/=20 x109/l and age >3 years as independent predictors for poor event-free survival event-free survival, while normal karyotype independently predicted inferior overall survival, event-free survival, and relapse-free survival. In conclusion, this study showed large differences in outcome within Myeloid leukemia of Down Syndrome patients and identified novel prognostic groups that predicted clinical outcome and hence may be used for stratification in future treatment protocols.
dc.relation.ispartof urn:issn:0390-6078
dc.title Normal karyotype is a poor prognostic factor in Myeloid Leukemia of Down Syndrome: a retrospective international study
dc.type Journal Article 2015-08-10T09:51:35Z
dc.language.rfc3066 en
dc.identifier.mtmt 2380959
dc.identifier.wos 000336253900022
dc.identifier.pubmed 23935021
dc.contributor.department SE/AOK/K/II. Sz. Gyermekgyógyászati Klinika
dc.contributor.institution Semmelweis Egyetem

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