Natural history of acquired immunodeficiency syndrome in hemophilic patients

During the 5-year period from 1981 to 1985, we have observed 8 cases of acquired immunodeficiency syndrome (AIDS) among our 85 patients with hemophilia A. Thus, the prevalence of AIDS with hemophilia A is 9.4% in our patient population. By utilizing stored serum or plasma samples dating back to 1978, antibody against HTLV-III was detected in all 8 cases with AIDS. Based on the time interval from the appearance of antibody to HTLV-III to the diagnosis of AIDS in these patients, the incubation period ranged from 27 months to 60 months, with a median of 36 months. Before the diagnosis of full-blown AIDS, all patients exhibited a variety of prodromal manifestations of non-specific nature, including weight loss, oral candidiasis, unexplained non-productive chronic cough, generalized lymphadenopathy, and thrombocytopenia lasting several months to several years. Serial T-lymphocyte subset studies were available in some patients during the HTLV-III seropositive period and showed progressive lymphopenia, depletion of T4 cells with an average absolute count of 94 +/- 128 per mm3 (mean +/- 1 S.D.), and a markedly reversed T4/T8 ratio of 0.26 +/- 0.19 (mean +/- 1 S.D.). These findings suggest that the incubation period of AIDS is considerably long and that prospective study of serial immunologic markers and HTLV-III markers may be warranted in hemophilic patients at risk.