ASH 2016: Children With Down Syndrome and ALL Fare as Well as Other Children Treated on ALL Consortium Protocols

Key Points

  • There was no difference in the rate at which children achieved complete remission after the first month of treatment (100% of children with Down syndrome vs 95.2% without).
  • With a median follow-up of 6.2 years, there was no statistically significant difference in 5-year rates of event-free survival (90.7% vs 83.7%), disease-free survival (90.7% vs 87.4%), or overall survival (91.8% vs 91.4%).
  • Patients with Down syndrome suffered more treatment-related mucositis (52% vs 12%), clots or bleeding (18.4% vs 8.2%), seizure (15.3% vs 4.7%), and infection (55.3% vs 41.3%).

Despite an elevated risk of toxicity from chemotherapy, children with Down syndrome and acute lymphoblastic leukemia (ALL) did not experience higher rates of relapse or treatment-related mortality compared with other children treated on Dana-Farber Cancer Institute ALL Consortium Protocols, according to research presented by Athale et al at the 58th American Society of Hematology (ASH) Annual Meeting & Exposition (Abstract 761).

“Without dose reductions or modifications, the Down syndrome patients did just as well as the non-Down syndrome patients,” said Lewis B. Silverman, MD, senior author of the abstract and Clinical Director of the Hematologic Malignancy Center at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center. “They were able to tolerate full-dose chemotherapy based on their risk group and did well despite biologic differences in their disease compared with other children’s disease.”

Down Syndrome and ALL

Children with Down syndrome are at increased risk for developing ALL, but the optimal therapy for this group of patients has not been established. Dr. Silverman notes that previous studies have shown that children with Down syndrome have an increased risk of complications of treatment. Some studies have also reported that they have higher rates of relapse and/or treatment-related mortality, resulting in lower rates of long-term cure. Although the Dana-Farber protocol has never modified treatment for children with Down syndrome, Dr. Silverman added, other protocols have made dose-adjustments to minimize side effects.

“There has been controversy in the field regarding how Down syndrome children do in terms of their prognosis compared with children who don’t have Down syndrome,” Dr.Silverman said. “We found that with our particular treatment approach, we’re not running into problems that others have reported.”

Study Findings

Researchers studied 1,286 diagnosed children and adolescents with ALL treated on the Dana-Farber Consortium protocols between 2000 and 2011 at 11 institutions in the United States, Canada, and Puerto Rico. Of these patients, 38 (3%) had Down syndrome. Among the findings:

  • There was no difference in the rate at which children achieved complete remission after the first month of treatment (100% of children with Down syndrome vs 95.2% without).
  • There were no treatment-related deaths among the Down syndrome patients.
  • With a median follow-up of 6.2 years, there was no statistically significant difference in 5-year rates of event-free survival (90.7% vs 83.7%), disease-free survival (90.7% vs 87.4%), or overall survival (91.8% vs 91.4%).
  • Patients with Down syndrome suffered more treatment-related mucositis (52% vs 12%), clots or bleeding (18.4% vs 8.2%), seizure (15.3% vs 4.7%), and infection (55.3% vs 41.3%).

As has been reported in other studies, researchers found that the Down syndrome patients were less likely than other patients to present with T-cell ALL (none in the Down syndrome group vs 11.7% in non–Down syndrome patients) and high hyperdiploidy (8.8% vs 25.1%). The former is considered a higher-risk form of ALL, whereas the latter is associated with a more favorable prognosis, Dr. Silverman said.

“The target toxicities that one needs to think about are infections and mucositis,” Dr. Silverman said. “With supportive care to try to prevent these complications, our overall recommendation is that you can treat children with Down syndrome the same as other children with ALL.”

The content in this post has not been reviewed by the American Society of Clinical Oncology, Inc. (ASCO®) and does not necessarily reflect the ideas and opinions of ASCO®.


Advertisement

Advertisement



Advertisement

By continuing to browse this site you permit us and our partners to place identification cookies on your browser and agree to our use of cookies to identify you for marketing. Read our Privacy Policy to learn more.