Current status of Swedish hemophiliacs. I. A demographic survey.

A comprehensive survey of the Swedish hemophilic population (hemophilia A and B) was undertaken in 1980. The total number of known hemophiliacs was 564. The ratio of hemophilia A to B was 4:1. The severe form constituted about 30% of each type. The incidence has remained fairly constant since 1957 and was 1.67 per year per 10,000 males born. The prevalence was 6.8 per 100,000 inhabitants. There was an increase in the prevalence in all counties but the increase varied considerably, 19-383%. The overall increase of hemophilia was 123%. Among mild cases the increase was 245%, whereas it was only 48% among the severe hemophiliacs. This skewness was attributed to better laboratory diagnoses and increased life expectancy. The proportion of mild cases increased from 35% in 1960 to 54% at the time of the present investigation. The situation was the reverse for the severe cases, a decrease from 45% to 30%. The mean and median ages among moderate and mild hemophiliacs deviated only little from the corresponding figure in the total population. Among severe cases, however, mean and median age were 8-13 years less than in the normal population.