Changes to this Summary (04/08/2015)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Added Corrigan et al. as reference 2.
Added text to state that in a genome-wide association study in the adolescent and young adult population, unique GATA3 polymorphisms were identified that strongly influence the susceptibility to leukemia in this population (cited Perez-Andreu et al. as reference 35).
Revised text to state that other reports confirm or do not confirm that 1% of neonatal blood spots tested positive for the ETV6-RUNX1 translocation, and methodological issues related to fluorescence in situ hybridization testing complicate interpretation of the initial 1% estimate (cited Olsen et al. and Kusk et al. as references 43 and 44, respectively).
Added Dreyer et al. as reference 12 and level of evidence 2A.
Added text to state that 6-mercaptopurine (6-MP) maintenance therapy adherence rates were significantly lower in Asian American and African American patients than in non-Hispanic white patients. A greater percentage of patients in these ethnic groups had adherence rates of less than 90%, which was associated with a 3.9-fold increased risk of relapse (cited Bhatia et al. as reference 72).
Added text to state that the results of one study of 40 patients with BCR-ABL-like ALL suggested that the adverse prognostic significance of this subtype may be abrogated when patients are treated with risk-directed therapy based on minimal residual disease (MRD) levels (cited Roberts et al. as reference 192 and level of evidence 2A).
Added Paganin et al. as reference 214.
Added Karsa et al. as reference 42.
Added text to state that studies conducted by the Children's Oncology Group (COG) have demonstrated significant differences in compliance with 6-MP amongst various racial and socioeconomic groups (cited Bhatia et al. as reference 44).
Revised text to state that a progressive increase in relapse was observed with decreasing adherence to 6-MP, with hazard ratios ranging between 4.0% to 5.7% for adherence rates ranging from 94.9% to 90%, 89.9% to 85%, and less than 85%. After adjusting for other prognostic factors, a progressive increase in relapse was observed with decreasing adherence to 6-MP.
Added text about a second study of adherence that was conducted in 298 children with ALL; an adherence rate of less than 90% was associated with increased relapse risk.
Added text to state that infants diagnosed within the first few months of life have a particularly poor outcome. In one study, patients diagnosed within 1 month of birth had a 2-year overall survival rate of 20%. Also added text to state that in another study, the 5-year event-free survival (EFS) for infants diagnosed at younger than 90 days was 16% (cited Dreyer et al. as reference 16 and level of evidence 2A).
Added text to state that infants have significantly higher relapse rates than older children with ALL and are at higher risk of developing treatment-related toxicity, especially infection. Also added text about the outcome of the COG AALL0631 trial for infants with ALL (cited Salzer et al. as reference 20).
Added text to state that on the COG P9407 trial, infants were treated with a shortened (46-week) intensive chemotherapy regimen. The 5-year EFS for infants with MLL translocations was 36%.
Added text to state that the COG P9407 trial of intensified chemotherapy reported a 70% 5-year EFS in infants without MLL translocations.
Added text about how RAS pathway mutations are common at relapse in B-precursor ALL patients (cited Irving et al. as reference 29).
Added text about the treatment and EFS results of the ALL-REZ BFM 2002 study.
Added text about the COG-AALL1331 trial as a treatment option under clinical evaluation for ALL in first relapse.
Added Chimeric Antigen Receptor (CAR) T-cell Therapy as a new subsection.
This summary is written and maintained by the PDQ Pediatric Treatment Editorial Board, which is editorially independent of NCI. The summary reflects an independent review of the literature and does not represent a policy statement of NCI or NIH. More information about summary policies and the role of the PDQ Editorial Boards in maintaining the PDQ summaries can be found on the About This PDQ Summary and PDQ NCI's Comprehensive Cancer Database pages.