Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia
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Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia. / Bohnstedt, C; Levinsen, M; Rosthøj, S; Zeller, B; Taskinen, M; Hafsteinsdottir, S; Björgvinsdóttir, H; Heyman, M; Schmiegelow, K; Nordic Society of Pediatric Hematology and Oncology (NOPHO).
In: Leukemia, Vol. 27, No. 4, 2013, p. 866-70.Research output: Contribution to journal › Journal article › Research › peer-review
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TY - JOUR
T1 - Physicians compliance during maintenance therapy in children with Down syndrome and acute lymphoblastic leukemia
AU - Bohnstedt, C
AU - Levinsen, M
AU - Rosthøj, S
AU - Zeller, B
AU - Taskinen, M
AU - Hafsteinsdottir, S
AU - Björgvinsdóttir, H
AU - Heyman, M
AU - Schmiegelow, K
AU - Nordic Society of Pediatric Hematology and Oncology (NOPHO)
PY - 2013
Y1 - 2013
N2 - 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.
AB - 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.
KW - Child
KW - Child, Preschool
KW - Down Syndrome
KW - Female
KW - Guideline Adherence
KW - Humans
KW - Male
KW - Physician's Practice Patterns
KW - Precursor Cell Lymphoblastic Leukemia-Lymphoma
U2 - 10.1038/leu.2012.325
DO - 10.1038/leu.2012.325
M3 - Journal article
C2 - 23138181
VL - 27
SP - 866
EP - 870
JO - Leukemia
JF - Leukemia
SN - 0887-6924
IS - 4
ER -
ID: 117364593