Current Trends Changing Patterns of Acquired Immunodeficiency Syndrome in Hemophilia Patients -- United States

The pattern of hemophilia-associated AIDS appears to be changing in that the number of cases may be stabilizing or declining, and the characteristics of new cases appear to be changing. As of April 1, 1985, CDC has received reports of 73 cases of hemophilia-associated acquired immunodeficiency syndrome (AIDS) among U.S. patients. The first case was diagnosed in 1981; eight cases were diagnosed in 1982; 13, in 1983; 45, in 1984; and six, thus far in 1985 (Figure 2). Four of these 73 had known risk factors for AIDS other than a coagulation disorder requiring treatment with commercial factor concentrates or cryoprecipitate. Patients with severe hemophilia A (hereditary factor VIII deficiency) continue to account for the majority (52 (71%)) of hemophilia-associated AIDS cases. Patients with mild or moderate hemophilia A account for an additional 13 (18%) cases. The remaining cases consist of three patients with hemophilia B (hereditary factor IX deficiency), three with von Willebrand's disease, one with an acquired inhibitor to factor VIII, and one with factor V deficiency. These patients resided in 27 different states. Cases reported per state ranged from one to nine (median two).

Ten patients had no documented use of blood products other than factor concentrates in the 5 years preceding their diagnoses. One patient with von Willebrand's disease, diagnosed in January 1985, had no documented use of blood products other than cryoprecipitate in the 3 years preceding diagnosis.

Sera from 29 (40%) of the 73 cases were obtained and tested by the Western blot method (1) for antibody to human T-lymphotropic virus type III/lymphadenopathy-associated virus (HTLV-III/LAV); 22 (76%) of the 29 were antibody-positive.

Of the opportunistic infections considered by CDC to be indicative of underlying cellular immune deficiency, Pneumocystis carinii pneumonia (PCP) remains the most common infection diagnosed in hemophilia-associated AIDS. Sixty-one (84%) of 73 patients had PCP alone or in combination with one or more other opportunistic infections.

Thirty-eight (52%) of the 73 hemophilia patients with AIDS have died. Seven (20%) of those still alive have survived 1 year or more since diagnosis; one (3%) has survived longer than 2 years.

Surveillance indicates the characteristics of recently diagnosed hemophilia-associated AIDS cases may be changing, and the number of new cases diagnosed by quarter may be stabilizing in this population. Ten of the 23 patients diagnosed since August 1, 1984, have disorders other than severe hemophilia A. This represents a change in proportion from earlier diagnosed cases (10 of 50 (p = 0.05)). During 1984, more cases of hemophilia-associated AIDS were diagnosed than in all previous years of surveillance. However, unlike the epidemic pattern for all AIDS, the number of hemophilia-associated AIDS cases in 1984 has not increased in each quarter (Figure 2). It is possible that a significant number of hemophilia-associated AIDS cases not yet reported to CDC have already been diagnosed at some time in 1984, and the temporal distribution of cases is subject to change with receipt of reports of such cases. However, preliminary results from a simulation of 1985 hemophilia/AIDS reporting indicate that the expected number and distribution of cases would not sufficiently change the 1984 hemophilia-AIDS epidemic pattern. Reported by Div of Host Factors, AIDS Br, Div of Viral Diseases, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: HTLV-III/LAV has been implicated as the causal agent of AIDS (2-5), and in the hemophilia population, commercial factor concentrates are suspected as the vehicle for transmission of the virus (6-8). Recently, exposure to HTLV-III/LAV through use of cryoprecipitate has been documented in studies of the seroprevalence (two of six tested) (9) and seroconversion (two of 11 seroconverting during a 1-year period) (10) in hemophilia patients using this product exclusively. The development of AIDS in three patients with von Willebrand's disease, one of whom had no documented blood product exposure other than cryoprecipitate and no other risk factor for AIDS, is further strong evidence to consider chronic use of cryoprecipitate a definite risk factor for AIDS. This may be especially true for those who are exposed to multiple donors (more than 80 per year). The magnitude of this risk may depend on geographic locality.

Trends in both the number and characteristics of recently reported hemophilia-associated AIDS appear to be changing. Patients with mild or moderate hemophilia and those with von Willebrand's disease tend to use significantly less clotting factor products in their disease therapy than do those with severe hemophilia and are more likely to be treated with products other than commercial factor concentrates. The recent increase in AIDS cases reported among persons with milder hemophilia may reflect earlier exposure of persons with severe hemophilia A to HTLV-III/LAV than of those with mild or moderate hemophilia or von Willebrand's disease. Continuous surveillance will be needed to monitor these trends. Physicians and other health-care personnel are encouraged to report suspected AIDS cases to CDC through their local or state health departments.

References

1. Tsang VC, Peralta JM, Simons AR. Enzyme-linked immunoelectrotransfer blot techniques (EITB) for studying the specificities of antigens and antibodies separated by gel electrophoresis. Methods Enzymol 1983;92:377-91.

2. Popovic M, Sarngadharan MG, Read E, Gallo RC. Detection, isolation, and continuous production of cytopathic retroviruses (HTLV-III) from patients with AIDS and pre-AIDS. Science 1984;224: 497-500.

3. Sarngadharan MG, Popovic M, Bruch L, Schupbach J, Gallo RC. Antibodies reactive with human T-lymphotropic retroviruses (HTLV-III) in the serum of patients with AIDS. Science 1984;224:506-8.

4. Gallo RC, Salahuddin SZ, Popovic M, et al. Frequent detection and isolation of cytopathic retroviruses (HTLV-III) from patients with AIDS and at risk for AIDS. Science 1984;224:500-3.

5. CDC. Antibodies to a retrovirus etiologically associated with acquired immunodeficiency syndrome (AIDS) in populations with increased incidences of the syndrome. MMWR 1984;33:377-9.

6. CDC. Update: acquired immunodeficiency syndrome (AIDS) in persons with hemophilia. MMWR 1984;33:589-91.

7. Ramsey RB, Palmer EL, McDougal JS, et al. Antibody to lymphadenopathy-associated virus in haemophiliacs with and without AIDS (letter). Lancet 1984;II:397-8.

8. Jason J, McDougal JS, Holman RC, et al. Lymphadenopathy-associated virus (LAV) antibody and its association with the immune status and factor concentrate usage of persons with hemophilia. JAMA (in press).

9. Koerper MA, Kaminsky LS, Levy JA. Differential prevalence of antibody to AIDS-associated retrovirus in haemophiliacs treated with factor VIII concentrate versus cryoprecipitate: recovery of infectious virus (letter). Lancet 1985;I:275.

10. McGrady G, Gjerset G, Kennedy S. Risk of exposure to HTLV-III/LAV and type of clotting factor used in hemophilia. Atlanta, Georgia: International Conference on AIDS, April 16, 1985.

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