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Physicians compliance during maintenance therapy in children with down syndrome and acute lymphoblastic leukemia.
MedLine Citation:
PMID:  23138181     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
Children with Down syndrome (DS) and acute lymphoblastic leukemia (ALL) have an inferior prognosis compared to non-DS ALL patients. We reviewed Methotrexate (MTX)/Mercaptopurine (6MP) maintenance therapy data for children with DS treated according to the NOPHO ALL92 or the NOPHO ALL2000 protocols between 1992 and 2007. The five year event-free survival (pEFS(5y)) for the 66 DS patients was inferior to the 2602 non-DS patients (0.50±0.07 vs 0.77±0.01, P<0.001). The 48 DS patients in 1st remission at the beginning of maintenance therapy had pEFS(10y) below that of the 522 non-DS control patients (pEFS(10y): 0.58, 95%-CI 0.43-0.77 vs 0.83, 95%-CI 0.80-0.86, P<0.0001). The DS patients received lower median doses of MTX (Median: 11.8 vs 15.4, P<0.0001) and 6MP (median: 45.6 vs 59.4, P<0.0001). In Cox regression analysis male gender, presence of DS, and high median maintenance therapy white blood cell levels (mWBC) were associated with increased risk for relapse. DS-ALL patients with mWBC above or below 3.5 × 10(9)/l (protocol target) had pEFS(10y) of 0.31 and 0.72 (P=0.02), and the mWBC hazard ratio for DS-ALL patients was 2.0 (P<0.0005). We conclude that insufficient treatment intensity during maintenance therapy of DS-ALL patients may contribute to their poor prognosis.Leukemia accepted article preview online, 9 November 2012; doi:10.1038/leu.2012.325.
Authors:
C Bohnstedt; M Levinsen; S Rosthøj; B Zeller; M Taskinen; S Hafsteinsdottir; H Björgvinsdóttir; M Heyman; K Schmiegelow
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-09
Journal Detail:
Title:  Leukemia : official journal of the Leukemia Society of America, Leukemia Research Fund, U.K     Volume:  -     ISSN:  1476-5551     ISO Abbreviation:  Leukemia     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-9     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8704895     Medline TA:  Leukemia     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Affiliation:
Pediatrics and Adolescent Medicine, The Juliane Marie Centre, The University Hospital Rigshospitalet, Copenhagen, Denmark.
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