|
|
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.
 Previous Article | Next Article 
Submitted October 24, 2007
Accepted December 18, 2007
Treatment and prognostic impact of transient leukemia in neonates with Down's syndrome
Jan-Henning Klusmann, Ursula Creutzig, Martin Zimmermann, Michael Dworzak, Norbert Jorch, Claudia Langebrake, Arnulf Pekrun, Katarina Macakova-Reinhardt, and Dirk Reinhardt*
Department of Pediatric Hematology and Oncology, Hannover Medical School, Hannover, Germany
Department of Pediatric Hematology and Oncology, University Childrens Hospital, Muenster, Germany
St. Anna Kinderspital, Childrens Cancer Research Institute, Vienna, Austria
Kinderklinik, Krankenanstalten Gilead, Bielefeld, Germany
University Hospital Hamburg-Eppendorf, University Hamburg, Hamburg, Germany
Prof-Hess-Kinderklinik, Klinikum Bremen-Mitte, Bremen, Germany
* Corresponding author; email: reinhardt.dirk{at}mh-hannover.de.
Approximately 10% of the neonates with Down's syndrome (DS) exhibit a unique transient leukemia (TL). Though TL resolves spontaneously in the majority of cases, 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, biological and treatment data of 146 patients with TL. 5-year overall survival (OS) and event free survival (EFS) were 85±3% and 63±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-yr EFS 52±12% vs. 28±11% (no treatment); p=0.02). Multivariate analysis demonstrated its favorable prognostic impact. 29 TL patients (23%) subsequently developed ML-DS. ML-DS patients with a history of TL had a significantly better 5-yr EFS (93±5%) than those without documented TL (71±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 #NCT 00111345.
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
A. C. Xavier, Y. Ge, and J. W. Taub
Down Syndrome and Malignancies: A Unique Clinical Relationship: A Paper from the 2008 William Beaumont Hospital Symposium on Molecular Pathology
J. Mol. Diagn.,
September 1, 2009;
11(5):
371 - 380.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
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]
|
|
|
|
|
