The goal of this study was to determine the long-term clinical outcome of Human Immunodeficiency Virus (HIV) infection in a group of HIV-seropositive hemophiliacs for whom the dates of seroconversion were known and to investigate whether the use of monoclonal antibody purified factor (high purity) concentrate and treatment with zidovudine may alter the effect of HIV infection in seropositive hemophiliacs. Twenty-eight hemophiliacs were followed for up to 9 years after seroconversion. In addition, 13 seropositive patients who elected to receive (high purity) factor VIII concentrate for up to 1.5 years were compared to a contemporaneous concurrent control group of 8 seropositive patients treated with intermediate purity factor VIII concentrate, and then both groups were followed for an additional 1.5 years while receiving zidovudine. The acquired immunodeficiency syndrome (AIDS) developed in 9 of 28 patients (33%) from 1 to 9 years following seroconversion. CD4 cell count declined at a rate of 13.5% per year for the cohort but for persons 25 years and above the rate was significantly higher (17.5 ± 9.2% per year vs. 9.5 ± 4.7%, mean ± SD, p < 0.05) than in those under 25. All three patients who had undergone splenectomy developed AIDS. Subjects treated with high purity concentrates, with the exception of one patient who developed the nephrotic syndrome secondary to amyloidosis, had stable CD4 cell counts. On the other hand, all patients receiving intermediate purity concentrates had a decline in CD4 cells, and the difference from initial levels was highly significant (p = 0.01). The CD4 cell concentrations in these patients were stable after treatment with zidovudine. We conclude that the incidence of AIDS in seropositive hemophiliacs more than 9 years after seroconversion is similar to that seen in other seropositive groups. CD4 cell counts declined more rapidly in those patients who were older at the time of seroconversion. Splenectomy may adversely affect the outcome of HIV infection. Individuals receiving high purity concentrate may have less immunosuppression than those receiving intermediate purity concentrates. Randomized controlled trials are needed to confirm these observations.
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