Natural history of acquired immunodeficiency syndrome in hemophilic patients

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Abstract
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.