EPIDEMIOLOGY OF HEMOPHILIA IN GREECE - AN OVERVIEW

Demographic data of the Greek haemophilia A and B population for the p eriod 1972-1993 were analyzed. prevalence at birth including known not -registered patients was calculated at 23.1 per 100,000 male births, H owever, the observed prevalence in 1993 was only 61% of the expected. Since 1975 the proportion of mild cases had significantly increased. A djusted by age, severity and HIV status reproductive fitness of haemop hiliacs was 0.62. Overall mortality was 2.6 times higher than in the g eneral population, but 7.9 times among patients with severe haemophili a and 16.4 among HIV(+) haemophiliacs. Fifty out of 78 deaths occurred among HN(+) patients and 28 of these were caused by AIDS. Inhibitor p atients did not show excess mortality due to bleeding. Cancer mortalit y was equal to normal, but the number of deaths from ischaemic heart d isease was 0.25 of the expected. Risk of death due to cerebral haemorr hage was 3.8 times higher in HN(+) haemophiliacs than in HIV(-).