Effects of HIV infection on age and cause of death for persons with hemophilia A in the United States

Because of changes in factor replacement therapy and in treatment of human immunodeficiency virus (HIV) infection, we examined death record data for p ersons with hemophilia A in the United States to evaluate effects of HIV in fection on age and causes of death. Multiple cause-of-death data from 1968 through 1998 were examined to assess death rates for persons with hemophili a A. ICD-9 coded causes of death from 1979 through 1998 were examined to as sess long-term trends. From 1979 through 1998, 4,781 deaths among persons w ith hemophilia A were reported, of which 2,254 (47%) had HIV-related diseas e listed as a cause of death. In the tate 1980s, mortality among persons wi th 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 year s in 1995-1998. In the period 1995-1998, the median age of hemophilia A dec edents with HIV-related disease was 33 years, compared to 72 years for thos e without HIV-related disease; the most frequently listed causes of death f or those without HIV-related disease were hemorrhagic and circulatory pheno mena; the most frequently listed for those with HIV-related disease were di seases of liver and the respiratory system, From 1995 to 1998, hemophilia A -associated deaths decreased by 41%, with a 78% decrease among those who ha d HIV-related disease. Although HIV infection has adversely effected mortal ity for persons with hemophilia A, the marked recent decrease in the death rate among persons with hemophilia A appears to reflect advances in care fo r those with HIV-related disease and is consistent with a decline in HIV mo rtality observed in the general population. Am. J. Hematol. 66:229-240, 200 1, Published 2001 Wiley-Liss, Inc.