PITTSBURGH – In the current era, children with Down syndrome who have standard risk B-cell precursor acute lymphoblastic leukemia have event-free and overall survival rates nearly as good as those of other children with standard-risk B–ALL, results of a Children’s Oncology Group study show.
Among 5,311 children enrolled in the COG AALL0331 trial, a study of combination chemotherapy for young patients with newly diagnosed ALL, the 5-year event-free survival (EFS) rate for children with Down syndrome was 86%, compared with 89% for children without Down syndrome (P = .025).
Neil Osterweil/MDedge News
Dr. Kelly Maloney
Although the differences in EFS and OS were significant, ”overall in this study, Down syndrome ALL had an excellent outcome that was similar to those patients without Down syndrome,” she said at the annual meeting of the American Society of Pediatric Hematology/Oncology.
The trial confirmed her group’s previous finding that there is a low rate of favorable cytogenetic features in patients with Down syndrome ALL; nonetheless, in the current study, 5-year continuous complete remission rates for standard-risk average, low, and high in patients with Down syndrome were similar to those for patients without Down syndrome, she said.
In the trial, patients were treated with a three-drug induction regimen, and following induction were assigned to standard-risk low, average, or high groups based on leukemia genetics and initial response to therapy.
Of the 5,311 children enrolled, 141 (2.7%) had Down syndrome, and these patients received risk-stratified therapy with additional supportive care guidelines, including leucovorin rescue after intrathecal methotrexate until maintenance. The care team strongly encouraged hospitalizations during high-risk blocks for this subgroup of patients until they experienced neutrophil recovery.