Severe aplastic anaemia in the Nordic countries: a population based study of incidence, presentation, course, and outcome.
OBJECTIVE
Incidence data for severe aplastic anaemia (SAA) in children are scanty and vary. Few population based studies have been reported. A retrospective and prospective study was conducted to determine the incidence and course of SAA.


METHODS
All children with a diagnosis of SAA in the Nordic countries from 1982 through 1993 were registered and have been followed up since 1987.


RESULTS
A total of 101 children were diagnosed with SAA. The mean annual child population was 4.31 million. A constant incidence of 1.95/million children/year was found: 2.4 for boys and 1.5 for girls. A non-significant increase of cases occurred from November to March. Possible aetiological agents were noted in 29%. The actuarial survival was 79% after one year and 68% after five years without significant difference between boys and girls.


CONCLUSIONS
The incidence of SAA in the Nordic countries remains stable with a preponderance among boys. SAA has still a high initial mortality and a risk of late deaths.
Clausen
N
N
Department of Paediatrics, University Hospital of Aarhus, Denmark.
Kreuger
A
A
Salmi
T
T
Storm-Mathisen
I
I
Johannesson
G
G
D000293:Adolescent; D000741:Anemia, Aplastic / Q000453:epidemiology* / Q000209:etiology; D002648:Child; D002675:Child, Preschool; D005260:Female; D005387:Finland / Q000453:epidemiology; D005500:Follow-Up Studies; D006801:Humans; D007055:Iceland / Q000453:epidemiology; D015994:Incidence; D007223:Infant; D008297:Male; D011379:Prognosis; D011446:Prospective Studies; D012189:Retrospective Studies; D012537:Scandinavian and Nordic Countries / Q000453:epidemiology; D012621:Seasons; D017678:Sex Distribution; D015996:Survival Rate