• HIV;
  • AIDS;
  • mortality;
  • hemophilia


Because of changes in factor replacement therapy and in treatment of human immunodeficiency virus (HIV) infection, we examined death record data for persons with hemophilia A in the United States to evaluate effects of HIV infection on age and causes of death. Multiple cause-of-death data from 1968 through 1998 were examined to assess death rates for persons with hemophilia A. ICD-9 coded causes of death from 1979 through 1998 were examined to assess long-term trends. From 1979 through 1998, 4,781 deaths among persons with hemophilia A were reported, of which 2,254 (47%) had HIV-related disease listed as a cause of death. In the late 1980s, mortality among persons with hemophilia A increased markedly, and the age-adjusted death rate peaked at 1.5 per 1,000,000 population in 1992. Median age at death decreased from 55 years in 1979–1982 to 40.5 years in 1987–1990, and increased to 46 years in 1995–1998. In the period 1995–1998, the median age of hemophilia A decedents with HIV-related disease was 33 years, compared to 72 years for those without HIV-related disease; the most frequently listed causes of death for those without HIV-related disease were hemorrhagic and circulatory phenomena; the most frequently listed for those with HIV-related disease were diseases of liver and the respiratory system. From 1995 to 1998, hemophilia A-associated deaths decreased by 41%, with a 78% decrease among those who had HIV-related disease. Although HIV infection has adversely effected mortality for persons with hemophilia A, the marked recent decrease in the death rate among persons with hemophilia A appears to reflect advances in care for those with HIV-related disease and is consistent with a decline in HIV mortality observed in the general population. Am. J. Hematol. 66:229–240, 2001. Published 2001 Wiley-Liss, Inc.