Childhood Acute Lymphoblastic Leukemia Treatment (PDQ®)–Health Professional Version

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Changes to This Summary (04/05/2018)

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.

Risk-Based Treatment Assignment

Added Tai et al. and Kahn et al. as references 76 and 77, respectively.

Added text to state that in a retrospective study of 373 patients treated at a single institution, body mass index (BMI) at diagnosis was not associated with minimal residual disease (MRD) at days 19 and 46, cumulative incidence of relapse, or event-free survival (EFS). Overall survival (OS) was lower in patients with a high BMI, primarily resulting from treatment-related mortality and inferior salvage after relapse (cited Eissa et al. as reference 89 and level of evidence 3iiA).

Revised text to state that notch pathway signaling is commonly activated by NOTCH1 and FBXW7 gene mutations in T-cell acute lymphoblastic leukemia (ALL); these are the most commonly mutated genes in pediatric T-cell ALL.

Added text about the prognostic significance of NOTCH1 and FBXW7mutations and how it may be modulated by genomic alterations in RASand PTEN, as reported by two study groups (cited Petit et al., Liu et al., and Trinquand et al. as references 114, 118, and 119, respectively).

Added text about the absolute risk of relapse that is associated with specific MRD levels and how the risk varies by genetic subtype (cited O'Connor et al. as reference 259).

Added text to state that the United Kingdom (UK)–ALL group reported that T-cell ALL patients with nondetectable end-induction MRD had excellent outcomes, while those with very high MRD levels at end of induction had a poor prognosis; however, for all other T-cell ALL patients, an association between end-induction MRD level and relapse risk was not found. Another study also indicated that MRD at a later time point may be more prognostically significant in T-cell ALL.

Postinduction Treatment for Specific ALL Subgroups

Added text about the results of the ALL-BFM-2000 trial of standard-risk patients who were randomly assigned to receive treatment with a single delayed-intensification phase of either standard intensity or reduced intensity (cited Schrappe et al. as reference 26).

Added text to state that compared with prednisone, dexamethasone has also been associated with a higher frequency of behavioral problems. In a randomized study of 50 patients aged 3 to 16 years who received maintenance chemotherapy, concurrent administration of hydrocortisone during dexamethasone pulses reduced the frequency of behavioral difficulties, emotional lability, and sleep disturbances (cited Warris et al. as reference 69).

Treatment of Relapsed Childhood ALL

Added Willasch et al. as reference 122.

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.

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