Risk Profiles in Childhood Acute Lymphoblastic Leukemia

Pediatric patients with B-cell–progenitor ALL and deleted or mutated IKZF1 had excessively poor outcomes.

The attainment of high cure rates for children with B-cell–progenitor acute lymphoblastic leukemia (ALL) is among the most important accomplishments of modern cancer care. Despite this achievement, treatment fails in about 20% of patients. To identify the molecular basis of therapeutic resistance and relapse, investigators evaluated gene-deletion and gene-mutation profiles in relation to outcomes in children with B-cell–progenitor ALL who were treated according to clinical protocols.

In a primary cohort of 221 high-risk children, the most common gene deletions were in CDKN2A/B, PAX5, and IKZF1 (46%, 32%, and 29% of patients, respectively). Overall, two thirds of the cohort had mutations in genes that encode transcriptional regulators of B-ly…

Reviewing Authors

Michael Douvas, MD

Michael E. Williams, MD

Disclosures

Consultant/Advisory BoardAbbVie; AstraZeneca; Beigene; Janssen; Pharmacyclics

Grant/Research SupportJanssen

Editorial BoardsAmerican Board of Internal Medicine (ABIM) Hematology Longitudinal Knowledge Approval Committee

Michael E. Williams, MD

Disclosures

Consultant/Advisory BoardAbbVie; AstraZeneca; Beigene; Janssen; Pharmacyclics

Grant/Research SupportJanssen

Editorial BoardsAmerican Board of Internal Medicine (ABIM) Hematology Longitudinal Knowledge Approval Committee