Long-term prognosis of children with Downs syndrome and leukaemia: a 34-year nation-wide experience

Background Although the characteristics of leukaemia in patients with Downʹs syndrome (DS) have been well documented, little is known about the long-term results of treatment. Method Retrospectively from 1968 to 1981 and prospectively from 1982 to 2002, the present authors collected data on every child with DS in Finland who had been diagnosed with leukaemia between 1968 and 1994. Results Forty-one children with DS had acute leukaemia: 28 had acute lymphoblastic leukaemia (ALL); and 13 had acute non-lymphoblastic leukaemia (ANLL). The median age of the subjects at diagnosis was 3.8 years (range = 0-15.9 years). Patients with ANLL were significantly younger (P = 0.001) and all patients under 2 years of age had ANLL. Out of the 28 patients with ALL, 23 (82%) entered primary remission, and of these 23 individuals, 10 remained alive and in continuous remission (CR) after a median of 11.6 years (range = 8.9-20.0 years). Out of the 13 patients with ANLL, five (38%) entered remission and four remained in CR after a median of 16.0 years (range = 9.1-19.2 years). Treatment -related toxicities were common: eight patients with ALL and two with ANLL died of septicaemia. Actuarial, event-free survival rates at 5 years were 53% and 43% for adequately treated subjects with ALL and ANLL, respectively. Conclusions Standard leukaemia chemotherapy is effective in patients with DS. However, because toxicities are unacceptably frequent, specific anti-leukaemia regimens are needed for subjects with DS design.