ArticleLeukemia2013
Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia.
Article in Leukemia, 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers, 2 of them syntheses that pooled it.
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Who cites it
15 citing papers in PubMed, 2 syntheses or guidelines pooled it, 36 citations in OpenAlex.
- PEG-asparaginase treatment regimens for acute lymphoblastic leukaemia in children: a network meta-analysis.The Cochrane database of systematic reviews · 2023Pooled it
- Pooled it
- Relapsed childhood acute lymphoblastic leukemia in the Nordic countries: prognostic factors, treatment and outcome.Haematologica · 2016Trial
- Outcomes in Children, Adolescents, and Young Adults With Down Syndrome and ALL: A Report From the Children's Oncology Group.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2024Article
- Maintenance Treatment in Acute Lymphoblastic Leukemia: A Clinical Primer.Indian journal of pediatrics · 2024Review
- Down syndrome-associated leukaemias: current evidence and challenges.Therapeutic advances in hematology · 2024Review
- Participation, challenges and needs in children with down syndrome during cancer treatment at hospital: a qualitative study of parents' experiences.Frontiers in rehabilitation sciences · 2023Article
- Minimal residual disease, long-term outcome, and IKZF1 deletions in children and adolescents with Down syndrome and acute lymphocytic leukaemia: a matched cohort study.The Lancet. Haematology · 2021Article
- Acute lymphoblastic leukemia and down syndrome: 6-mercaptopurine and methotrexate metabolites during maintenance therapy.Leukemia · 2021Article
- Non-infectious chemotherapy-associated acute toxicities during childhood acute lymphoblastic leukemia therapy.F1000Research · 2017Review
- The biology, pathogenesis and clinical aspects of acute lymphoblastic leukemia in children with Down syndrome.Leukemia · 2016Review
- Childhood Acute Lymphoblastic Leukemia: Progress Through Collaboration.Journal of clinical oncology : official journal of the American Society of Clinical Oncology · 2015Review
- Mercaptopurine/Methotrexate maintenance therapy of childhood acute lymphoblastic leukemia: clinical facts and fiction.Journal of pediatric hematology/oncology · 2014Review
- Clinical and genetic features of pediatric acute lymphoblastic leukemia in Down syndrome in the Nordic countries.Journal of hematology & oncology · 2014Article
- Article
Corrections and comments
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Authors and funding
10 authors at 6 institutions in 5 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Children with Down syndrome (DS) and acute lymphoblastic leukemia (ALL) have an inferior prognosis compared with non-DS ALL patients. We reviewed methotrexate (MTX)/mercaptopurine (6MP) maintenance therapy data for children with DS treated according to the Nordic Society of Pediatric Hematology and Oncology (NOPHO) ALL92 or the NOPHO ALL2000 protocols between 1992 and 2007. The 5-year event-free survival probability (pEFS(5 yr)) 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 first remission at the beginning of maintenance therapy had pEFS(10 yr) below that of the 522 non-DS control patients (pEFS(10 yr): 0.58 (95% confidence interval (CI) 0.43-0.77) vs 0.83 (95% CI 0.80-0.86), respectively (P<0.0001)). The DS patients received lower median doses of MTX (median: 11.8 vs 15.4 (P<0.0001)) and 6MP (median: 43.6 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(10 yr) 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.
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