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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 less than 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.(ABSTRACT TRUNCATED AT 250 WORDS)

The once-isolated world of the residential treatment center has been altered by the broad social and cultural changes of recent years. New values, especially as introduced by younger staff whose attitudes are often closer to their clients than to their administrators, have created new problems. This paper points to the need for a sensitive professional response, and the ability to embrace change and put it to new therapeutic uses.

We examined 35 patients with hemophilia to determine if there was an association between impaired cell-mediated immunity and the amount of factor concentrate use. There was a significant negative relationship between the logarithm of the helper-suppressor ratio and the logarithm of concentrate use determined over the previous one year, five years, and total lifetime. Similarly, the presence of splenomegaly was significantly associated with the logarithm of concentrate use for each time interval. Hypergammaglobulinemia, anergy, and lymphadenopathy were present in a high proportion of patients. Repeated study of 30 of these patients at eight to 14 months showed no significant changes in their T-cell subsets. At follow-up, 16 patients had lymphadenopathy with or without splenomegaly and four had splenomegaly alone. No significant associations between concentrate use during the study period and changes in T-cell subsets or clinical condition were found.

Early reports suggested that hemophiliacs with factor IX deficiency (Christmas Disease) may be at less risk for developing the acquired immunodeficiency syndrome (AIDS) than patients with classic hemophilia. We evaluated 12 factor IX deficient patients for clinical and immunologic abnormalities related to infection with the human immunodeficiency virus (HIV). Antibody to HIV was not detected in these patients prior to 1982. By 1985, 66 percent (eight of 12) patients were seropositive. All three concentrates available commercially before 1985 were associated with seropositivity. Furthermore, seropositive hemophiliacs had received on average significantly more factor IX concentrate than seronegative hemophiliacs (27,825 +/- 17,976 (S.D.) versus 1,250 +/- 1,500 factor units/year, (p less than 0.02). Half of the seropositive individuals had generalized lymphadenopathy with splenomegaly. Two seropositive patients have developed AIDS, one with cryptococcal meningitis and another with a large cell immunoblastic lymphoma. Infection with HIV has occurred with high frequency in hemophiliacs who received unmodified factor IX concentrates.

Twenty homosexual men who reported having sexual contact with homosexual men who had the acquired immunodeficiency syndrome (AIDS) or the AIDS-related complex were examined to determine their clinical status, immunologic profiles, and the presence of antibody to the human T-cell lymphotropic virus type III (HTLV-III). Of the 20, eight men had one or more signs or symptoms of the AIDS-related complex and 12 were asymptomatic. Antibodies to HTLV-III were present in 14 (70%) of 20 of the sexual contacts as compared with four (10%) of 40 healthy homosexuals without known contact with a patient who had AIDS (P less than .0001). Seropositive contacts had significantly higher mean counts of suppressor lymphocytes and lower helper: suppressor ratios (P less than .05 and .005, respectively) and higher serum levels of IgG than seronegative contacts (P less than .05). It was not possible to determine significant differences in sexual practices, drug use, length of relationship, or numbers of different sexual partners between symptomatic and asymptomatic contacts or between seropositive and seronegative contacts in this study.

Patients with the acquired immunodeficiency syndrome (AIDS) are susceptible to a variety of opportunistic pathogens which require intact cellular immunity for control and eradication. We evaluated interleukin 1 and 2 production in 12 homosexual men without AIDS but with evidence of altered cell-mediated immunity and serologic evidence of infection with human T-cell lymphotrophic virus type III (HTLV-III), the etiologic agent of AIDS and found production of both factors diminished compared to heterosexual controls. Therafectin (SM-1213) is a new agent which selectively activates macrophages and stimulated interleukin 1 production in vitro. Therafectin was administered to these same 12 patients in a double-blind, placebo controlled trial. We failed to find any significant changes in their immunologic status including interleukin 1 or 2 production.