期刊
LEUKEMIA
卷 30, 期 1, 页码 32-38出版社
SPRINGERNATURE
DOI: 10.1038/leu.2015.199
关键词
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资金
- Dutch Cancer Society (KWF) [EMCR 2007-3718, KUN 2009-4298]
- Sophia Foundation for Scientific Research (SSWO) [658]
- Paediatric Oncology Foundation Rotterdam
- European Union's Seventh Framework Program (FP7) under the project European Network for Cancer research in Children and Adolescents (ENCCA) [HEALTH-F2-2011-261474]
- Center for Translational Molecular Medicine BioChip program
- National Health and Medical Research Council
- Tour de Cure Foundation in Australia
- Polpharma Scientific Foundation
- Anniversary of the Austrian National Bank (ONB) [14133]
- Italian Association for Cancer Research (AIRC)
- Czech Ministry of Health [NT 12397-4, NT 13170-4]
- Fondazione Cariplo
- MIUR
- Leukaemia AMP
- Lymphoma Research (LLR)
Deletions in IKZF1 are found in similar to 15% of children with B-cell precursor acute lymphoblastic leukemia (BCP-ALL). There is strong evidence for the poor prognosis of IKZF1 deletions affecting exons 4-7 and exons 1-8, but evidence for the remaining 33% of cases harboring other variants of IKZF1 deletions is lacking. In an international multicenter study we analyzed the prognostic value of these rare variants in a case-control design. Each IKZF1-deleted case was matched to three IKZF1 wild-type controls based on cytogenetic subtype, treatment protocol, risk stratification arm, white blood cell count and age. Hazard ratios for the prognostic impact of rare IKZF1 deletions on event-free survival were calculated by matched pair Cox regression. Matched pair analysis for all 134 cases with rare IKZF1 deletions together revealed a poor prognosis (P < 0.001) that was evident in each risk stratification arm. Rare variant types with the most unfavorable event-free survival were DEL 2-7 (P = 0.03), DEL 2-8 (P = 0.002) and DEL-Other (P < 0.001). The prognosis of each type of rare variant was equal or worse compared with the well-known major DEL 4-7 and DEL 1-8 IKZF1 deletion variants. We therefore conclude that all variants of rare IKZF1 deletions are associated with an unfavorable prognosis in pediatric BCP-ALL.
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