Acquired Immunodeficiency Syndrome Among Patients Attending Hemophilia Treatment Centers and Mortality Experience of Hemophiliacs in the United States

Abstract

The acquired immunodeficiency syndrome (AIDS) was first recognized among hemophiliacs in 1982. The authors have conducted investigations to determine the onset and incidence of AIDS among hemophiliacs and to determine trends in hemophilia mortality since the introduction of clotting-factor concentrates in the late 1960s. A survey of United States hemophilia treatment centers, supported by the Centers for Disease Control and the National Hemophilia Foundation, defined a population of hemophiliacs which was monitored for AIDS cases through June 1984. Death reports from the United States Vital Statistics System and from the hemophilia treatment center survey provided mortality trends for 1968-1979 and for 1978-1982, respectively. The results of these investigations demonstrate the following points. 1) The AIDS epidemic is a new and important cause of illness and mortality among hemophiliacs, although a very low incidence of AIDS among hemophiliacs prior to 1982 cannot be ruled out. 2) The AIDS cases who attended the surveyed hemophilia treatment centers were distributed throughout the United States and were older than hemophilia treatment center patients without AIDS. AIDS cases also used more lyophilized clotting-factor concentrate, but only a small number of cases were reported with this information. 3) Improved care for hemophilia, including the use of clotting-factor concentrates, dramatically reduced hemophilia mortality rates during the 1970s. 4) In 1982, hemorrhage was the major cause of death among hemophiliacs. Deaths from non-alcoholic liver disease were also increased. AIDS incidence among hemophilia treatment center attendees was stable at 0.6 cases per 1,000 hemophilia treatment center attendees per year during 1982 and 1983 but increased sharply to 5.4 cases per 1,000 during the first quarter of 1984.

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

“…In the 1960s and 1970s, the introduction of factor replacement therapy, the development of comprehensive hemophilia treatment centers, and the implementation of home care for this population decreased the number and proportion of deaths due to hemorrhage [7], dramatically increasing their median age at death [33,34]. In this study, these perturbations are underscored by the disparity in the median age at death in 1995-1998 for persons with hemophilia A between those with HIV-related disease (33 years) and those without HIV-related disease (72 years).…”

“…By 1982, 73% of persons with hemophilia A treated at U.S. hemophilia treatment centers received lyophilized concentrates, with the majority receiving their therapy almost exclusively at home [7]. Home infusion enabled earlier treatment of hemorrhages and markedly reduced their severity.…”

Mortality and causes of death in Dutch haemophiliacs, 1973–86

“…Although this flgure was still markedly below that of the general population, he also observed that the death rates for the younger patients in the last 10 years of his study were approaching those of the general Swedish population. Johnson et al (1985) found the age-adjusted mortality among United States haemophiliacs during 1978-82 to be 2-3 times äs high äs that of the general male population. Some deaths may have been missed, however, since this result was based on reported deaths and visits to haemophilia centres, rather than on a cohort study.…”

Multiple-dose pharmacokinetics of oral zidovudine in hemophilia patients with human immunodeficiency virus infection

“…A pharmacologic consideration for the use of ZDV in hemophilia patients with HIV disease is the presence of chronic hepatitis among many of these patients and the potential for impaired drug metabolism (2,3,6,(8)(9)(10). A recent report on the disposition of a single oral dose of ZDV in patients with hemophilia described considerable variability with regard to the concentrations in plasma attained (12).…”