SEVERE APLASTIC-ANEMIA IN THE NORDIC COUNTRIES - A POPULATION-BASED STUDY OF INCIDENCE, PRESENTATION, COURSE, AND OUTCOME

Purpose-Incidence data for severe aplastic anaemia (SAA) in children a re scanty and vary. Few population based studies have been reported. A retrospective and prospective study was conducted to determine the in cidence and course of SAA. Patients and methods-Ah children with a dia gnosis of SAA in the Nordic countries from 1982 through 1993 were regi stered and have been followed up since 1987. Results-A total of 101 ch ildren were diagnosed with SAA. The mean annual child population was 4 .31 million. A constant incidence of 1.95/million children/year was fo und: 2.4 for boys and 1.5 for girls. A non-significant increase of cas es occurred from November to March. Possible aetiological agents were noted in 29%. The actuarial survival was 79% after one year and 68% af ter five years without significant difference between boys and girls. Conclusion The incidence of SAA in the Nordic countries remains stable with a preponderance among boys. SAA has still a high initial mortali ty and a risk of late deaths.