High risk acute lymphoblastic leukemia in Denmark

Abstract
In a retrospective study of acute lymphoblasic leukemia (ALL) in Denmark from 1973 to 1981, 95/267 children below 15 years of age fulfilled at least one of the following high risk (HR) criteria: WBC > 50 × 109/1, mediastinal mass, meningeal leukemia, extramedullary leukemia, T-ALL, B-ALL and congenital leukemia. Significantly more of the children in HR had considerable hepato- and/or splenomegaly compared with children in the standard risk (SR) and intermediate risk (IR) groups. Hemoglobin values above 6.0 mmol/l were found in 35% of HR children and in 9% of children in SR and IR (p < 0.0005). Treatment protocols varied. The percentage achieving complete remission (CR) was 93.7. Median relapse free survival (RFS) was 18 months. Twenty-nine children (32.5%) have been in CCR for 24–114 months as of July 1983. Initial WBC was the only independent prognostic parameter. Of children with initial WBC < 50 × 109/l, 62% remained in CCR compared to 15% with WBC > 50 × 109/l. Treatment protocols employed before July 1977 were significantly inferior to protocols employed during the last half of the study period. Fifty-seven patients relapsed most frequently in the bone marrow. In 38.5% of the relapses, the central nervous system (CNS) was involved. When the CNS prophylaxis was initiated early during induction treatment, significantly fewer CNS recurrences arose than when the CNS prophylaxis was started after remission had been induced. Acute lymphoblastic leukemia, children, high risk, prognosis.