Children with NCI Standard Risk Acute Lymphoblastic Leukemia (ALL) and TEL-AML1 or Favorable Chromosome Trisomies Are Almost Certain to Be Cured with Graduated Intensity Therapy: Results of the CCG - 1991 Study.

Three thousand fifty four children with NCI standard-risk (SR) ALL were enrolled on CCG-1991; 2075 non-T ALL eligible patients were randomized and began treatment with IT cytarabine, vincristine, dexamethasone, and pegylated asparaginase. Bone marrow status was assessed at Day 7, 14, and 28 of Induction. Slow early responders (Day 7/14 M3-M3 and M3-M2; or M2 at Day 28 Induction) and patients with unfavorable cytogenetics were non-randomly assigned to a COG augmented BFM therapy regimen (N Engl J Med 1998; 338:1663).Thirteen hundred and thirty patients were analyzed for the presence of trisomy 4 and trisomy 10 (DT); and 1041 patients were evaluated for the presence of TEL/AML1 (ETV6/RUNX1) (TEL) fusion transcript. Twenty four percent of patients were positive for DT, and 41% were positive for TEL. Among the randomized subset, 23.8% had DT, and 40.8% had TEL. Six year event-free survival (EFS) and overall survival (OS) rates are given in the tables below for all patients and also separately for the randomized patients, by DT and TEL status. There was a significant different in EFS and OS between patients that were DT+ vs. DT- and also between patients that were TEL+ vs. TEL-.The overall 6-year EFS and OS for all patients with B-lineage ALL are 88.1±1.1% and 94.7±0.8%; and for the randomized patients they are 89.4±2.4% and 95.9±0.8%. We conclude that COG risk-adapted therapy is curative for patients with NCI-SR criteria and DT and/or TEL.

No relevant conflicts of interest to declare.