Leukemia With IKZF₁ Deletion Benefits From Prolonged Maintenance Therapy

Prolonged maintenance therapy for IKZF₁del improved five-year cumulative risk for relapse and event-free survival
acute lymphoblastic leukemia
acute lymphoblastic leukemia

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Medically Reviewed By:
Mark Arredondo, M.D.

MONDAY, July 31, 2023 (HealthDay News) -- For children with acute lymphoblastic leukemia (ALL), cases with IKZF₁ deletion (IKZF₁del) benefit from prolonged maintenance therapy, according to a study published online July 17 in the Journal of Clinical Oncology.

Noting that IKZF₁del ALL showed poor response to the ALL10 protocol in children in the largest minimal residual disease-defined medium-risk group, accounting for a high number of overall relapses, Rob Pieters, M.D., Ph.D., from the Princess Maxima Center for Pediatric Oncology in Utrecht, Netherlands, and colleagues compared results for the ALL11 protocol, which prolonged therapy for IKZF₁del from two to three years, with the ALL10 protocol. A total of 819 patients with ALL were enrolled in ALL11 and stratified as in ALL10.

The researchers found that in ALL11, the five-year overall survival, event-free survival (EFS), cumulative risk for relapse (CIR), and death in complete remission were 94.2, 89.0, 8.2, and 2.3 percent, respectively. Five-year CIR improved to 10.8 percent from 23.4 percent, and EFS improved to 87.1 percent from 72.3 percent with prolonged maintenance. In a landmark analysis at two years from diagnosis, there was a 2.9-fold reduction of CIR (25.6 to 8.8 percent) and improvement in EFS (74.4 to 91.2 percent). Reduced therapy did not negate the five-year outcomes for ETV6::RUNX, Down syndrome, or poor prednisone responders.

"Children with IKZF₁del ALL seem to benefit from a third year of therapy, suggesting to change therapy accordingly for this class of patients," the authors write.

Several authors disclosed ties to the pharmaceutical industry.

Abstract/Full Text

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