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Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®)

Health Professional Version
Last Modified: 10/29/2014

Changes to this Summary (10/29/2014)

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.

General Information About Childhood Acute Lymphoblastic Leukemia (ALL)

Added Smith et al. as reference 1.

Revised text to state that for ALL, the 5-year survival rate has increased over the same time from 60% to approximately 90% for children younger than 15 years and from 28% to more than 75% for adolescents aged 15 to 19 years.

Added text to state that carriers of a constitutional Robertsonian translocation that involves chromosomes 15 and 21 are specifically and highly predisposed to developing iAMP21 ALL (cited Li et al. as reference 14).

Added Buitenkamp et al. as reference 21 and Migliorini et al. as reference 32.

Risk-Based Treatment Assignment

Added text to state that in a large retrospective study of patients with Down syndrome and ALL, age younger than 6 years, white blood cell count of less than 10,000/µL, and the presence of the ETV6-RUNX1 fusion were independent predictors of favorable event-free survival (EFS); failure to achieve remission and treatment-related mortality are also higher in patients with Down syndrome (cited Buitenkamp et al. as reference 54).

Revised text to state that NOTCH1-activating gene mutations occur in approximately 50% to 60% of T-cell ALL cases, and FBXW7-inactivating gene mutations occur in approximately 15% of cases, with the result that approximately 60% of cases have Notch pathway activation by mutations in at least one of these genes (cited Gallo Llorente et al. as reference 86).

Added Fogelstrand et al. as reference 90.

Added text to state that another study indicated that the early T-cell precursor ALL subgroup had nonsignificantly inferior 5-year EFS compared with non–early T-cell precursor cases (76% vs. 84%); further study in additional patient cohorts is needed to firmly establish the prognostic significance of early T-cell precursor ALL (cited Patrick et al. as reference 104).

Added text to state that it does not appear that the presence of secondary cytogenetic abnormalities, such as deletion of ETV6 (12p) or CDKN2A/B (9p), impacts the outcome of patients with the ETV6-RUNX1 fusion (cited Enshaei et al. and Barbany et al. as references 137 and 138, respectively).

Revised text about the intrachromosomal amplification of chromosome 21 (iAMP21) and added text about the results of trials from the United Kingdom-ALL clinical trials group and the Children's Oncology Group (COG) regarding the prognostic significance of the presence of iAMP21 in patients with ALL (cited Heerema et al., Moorman et al., and Harrison et al. as references 165, 166, and 167, respectively).

Added Olsson et al. as reference 177.

Added text about the prevalence and prognostic significance of the ERG deletion (cited Zaliova et al. and Clappier et al. as references 178 and 179, respectively).

Added text about the NCI-2014-00712/AALL1231 trial and the criteria that the COG uses to assign risk categories.

Postinduction Treatment for Childhood ALL

Added text about the results from the AIEOP ALL-BFM-2000 study, which found that no statistically significant difference was found for disease-free survival in patients with high-risk features who received a transplant versus those who did not, after adjusting for waiting time to hematopoietic stem cell transplantation (HSCT) (cited Conter et al. as reference 34 and level of evidence 2Dii).

CNS-Directed Therapy for Childhood ALL

Added text about the NCI-2014-00712/AALL1231 trial as a presymptomatic CNS therapy option under clinical evaluation.

Postinduction Treatment for Specific ALL Subgroups

Added text about the NCI-2014-00712/AALL1231 trial as a treatment option under clinical evaluation for T-cell ALL.

Added text about the NCI-2014-00712/AALL1231 trial as a treatment option under clinical evaluation for adolescent and young adult patients with ALL.

Treatment of Relapsed Childhood ALL

Added text to state that an analysis of data from the Center for International Blood and Marrow Transplant Research (CIBMTR) on 27 Down syndrome patients with ALL who underwent HSCT between 2000 and 2009 indicated that hematopoietic recovery, graft-versus-host disease (GVHD) and transplant-related mortality were within the expected range for non–Down syndrome ALL patients; in that series, relapse rather than transplant toxicity was the primary cause of treatment failure (cited Hitzler et al. as reference 20 and level of evidence 3iiiA).

Added text to state that in the ALL-REZ BFM P95/96 study from the BFM group, end-reinduction minimal-residual disease (MRD) significantly predicted outcome of children with intermediate-risk relapsed B-cell ALL treated with chemotherapy alone in second complete remission.

Added Balduzzi et al. as reference 67 and Sanchez-Garcia et al. as reference 73 and level of evidence 3iiB.

Added text about one report of 13 ALL patients with high MRD who received an additional cycle of chemotherapy in an attempt to lower MRD before proceeding to HSCT.

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.