Survival in a cohort of patients with haemophilia at the haemophilia care center in Vienna, Austria, from 1983 to 2006

Abstract

Survival of patients with haemophilia is still a relevant issue of great interest. A survival analysis was conducted among 226 patients with haemophilia A and B (128 severe haemophiliacs), who were treated at the haemophilia care centre in Vienna. Information on mortality in our patient cohort was obtained from the Austrian Central Death Register. Overall, 96 of a total of 226 patients (42.5%) died between 1983 and 2006; 37 patients (38.5%) died due to HIV-infection, 15 due to HCV infection, 15 due to bleeding (15.6%, respectively) and 29 (30.2%) due to various other causes. The mortality of HIV-positive patients was 74.3% (n = 55) and that of HCV-positive patients was 40.4% (n = 55) in the analysed period. The patient mortality rates were compared with those of the general Austrian male population following adjustment for age and calendar period. We found that the cumulative relative survival of all patients was 0.694 (95% CI 0.614-0.767). The cumulative relative survival of patients with severe haemophilia (FVIII or IX level < or =1%) was 0.489 (0.394-0.579), but was normal (0.986; 95% CI 0.858-1.082) for patients with mild or moderate haemophilia (FVIII or IX level 2-50%). The survival rate was lowest in HIV-positive patients (0.287; 95% CI 0.186-0.398), but was also decreased to 0.874 (0.776-0.951) in HIV-negative patients. It can, therefore, be concluded that the survival of patients with severe haemophilia is still decreased compared to those with non-severe haemophilia and the general male population, regardless of HIV-infection.