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Blood, 15 March 2008, Vol. 111, No. 6, pp. 2991-2998. Prepublished online as a Blood First Edition Paper on January 8, 2008; DOI 10.1182/blood-2007-10-118810. This Article Full Text Full Text (PDF) Supplemental Appendix and Table All Versions of this Article: blood-2007-10-118810v1 111/6/2991    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Klusmann, J.-H. Articles by Reinhardt, D. Search for Related Content PubMed PubMed Citation Articles by Klusmann, J.-H. Articles by Reinhardt, D. Related Collections Free Research Articles Clinical Trials and Observations Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS Treatment and prognostic impact of transient leukemia in neonates with Down syndrome Jan-Henning Klusmann1, Ursula Creutzig2, Martin Zimmermann1, Michael Dworzak3, Norbert Jorch4, Claudia Langebrake5, Arnulf Pekrun6, Katarina Macakova-Reinhardt1, and Dirk Reinhardt1 1 Department of Pediatric Hematology and Oncology, Medical School Hannover, Hannover, Germany; 2 Department of Pediatric Hematology and Oncology, University Children's Hospital, Muenster, Germany; 3 St Anna Kinderspital and Childrens Cancer Research Institute, Vienna, Austria; 4 Kinderklinik Krankenanstalten Gilead, Bielefeld, Germany; 5 University Hospital Hamburg-Eppendorf, University Hamburg, Hamburg, Germany; and 6 Prof-Hess-Kinderklinik, Klinikum Bremen-Mitte, Bremen, Germany Approximately 10% of the neonates with Down syndrome (DS) exhibit a unique transient leukemia (TL). Though TL resolves spontaneously in most patients, early death and development of myeloid leukemia (ML-DS) may occur. Prognostic factors as well as treatment indication are currently uncertain. To resolve that issue, we prospectively collected clinical, biologic, and treatment data of 146 patients with TL. The 5-year overall survival (OS) and event-free survival (EFS) were 85% plus or minus 3% and 63% plus or minus 4%, respectively. Multivariate analysis revealed a correlation between high white blood cell (WBC) count, ascites, preterm delivery, bleeding diatheses, failure of spontaneous remission, and the occurrence of early death. Treatment with cytarabine (0.5-1.5 mg/kg) was administered to 28 patients with high WBC count, thrombocytopenia, or liver dysfunction. The therapy had a beneficial effect on the outcome of those children with risk factors for early death (5-year EFS, 52% ± 12% vs 28% ± 11% [no treatment]; P = .02). Multivariate analysis demonstrated its favorable prognostic impact. A total of 29 (23%) patients with TL subsequently developed ML-DS. Patients with ML-DS with a history of TL had a significantly better 5-year EFS (91% ± 5%) than those without documented TL (70% ± 4%), primarily due to a lower relapse rate. A history of TL may therefore define a lower-risk ML-DS subgroup. This study was registered at www.clinicaltrials.gov as no. NCT 00111345. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: I. Ganmore, G. Smooha, and S. Izraeli Constitutional aneuploidy and cancer predisposition Hum. Mol. Genet., April 15, 2009; 18(R1): R84 - R93. [Abstract] [Full Text] [PDF] S. Malinge, S. Izraeli, and J. D. Crispino Insights into the manifestations, outcomes, and mechanisms of leukemogenesis in Down syndrome Blood, March 19, 2009; 113(12): 2619 - 2628. [Abstract] [Full Text] [PDF] K. R. Rabin and J. A. Whitlock Malignancy in Children with Trisomy 21 Oncologist, February 1, 2009; 14(2): 164 - 173. [Abstract] [Full Text] [PDF]

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