Changes in the life expectancy of patients with severe haemophilia A in Finland in 1930-79

Abstract

Important advances have been made in the treatment of haemophilia during the past 30 years. We have analysed the data of all the known 163 patients with severe haemophilia A living in Finland in 1930-79 in order to study changes in the prognosis of severe haemophilia A. During the period of 50 years the mean age at death of the patients has increased from 7.8 years in 1930-39 to 25.5 years in 1970-79 and the annual death rate has markedly decreased in all age groups. The decline has been greatest in patients under 10 years of age. In this age group the annual death rate decreased from over 50 per thousand in 1930-39 and 1940-49 to 4.8 per thousand in 1970-79. The prognosis of patients with inhibitors has remained poor, however. Five of the six deaths during the last decade occurred in patients with inhibitors. The overall annual death rate of patients without inhibitors was only 1.2 per thousand in 1970-79, suggesting that at the present time the life expectancy of patients who do not develop inhibitors does not markedly differ from that of the general male population.