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Surveillance of Hemophilia-Associated Acquired Immunodeficiency Syndrome

As of September 15, 1986, a total of 238 cases of hemophilia-associated acquired immunodeficiency syndrome (AIDS) have been reported to CDC through state health departments, hemophilia treatment centers (HTCs), and physicians. Of the 238 patients, 212 (89%) had hemophilia A (coagulation factor VIII deficiency); 16 (7%), hemophilia B (factor IX deficiency); seven (3%), von Willebrand's disease; two, an acquired inhibitor (antibody) to factor VIII; and one, a factor V deficiency. All but seven (3%) of the patients were male. Thirteen patients were known to have had other risk factors for AIDS in addition to a hematologic disease. The 238 patients resided in 38 states; almost half lived in California, New York, Pennsylvania, New Jersey, or Missouri. The total number of cases represents a cumulative incidence of 1.6 cases of AIDS/100 hemophiliacs in the United States (1).

The first AIDS patient with underlying coagulation disorders was diagnosed as having Pneumocystis carinii pneumonia in 1981. Later it was recognized that this patient had AIDS. Since then, the number of hemophilia-associated AIDS cases has increased each year. The reported number of cases among hemophiliacs does not appear to be increasing at an exponential rate (Figure 1); however, in 1985, 92% of persons with hemophilia A and 52% of those with hemophilia B in a U.S. hemophilia cohort had antibodies to human T-lymphotropic virus type III/lymphadenopathy-associated virus (HTLV-III/LAV)*, suggesting exposure to the virus or to virus particles (2). HTLV-III/LAV seropositivity in this cohort was associated with declining Thelper lymphocyte numbers and with declining Thelper-to-Tsuppressor cell ratios. Because of these high rates of seroprevalence and immunology findings, concern had been expressed that the recent incidence of hemophilia-associated AIDS may be misleadingly low because of a decline in reporting.

To determine the completeness of reporting, the Division of Host Factors (DHF), Center for Infectious Diseases, CDC, and the National Hemophilia Foundation (NHF) surveyed all United States HTCs, local NHF chapters, and physicians known to have patients with hemophilia (3). On May 14, 1986, each HTC/physician was sent a list of persons with hemophilia-associated AIDS according to DHF records as of May 1, 1986. Since patients' names are not used at DHF, cases were identified only by the patient's date of birth, the date of diagnosis, and the nature of the AIDS diagnosis. The HTCs/physicians were asked to add to this list any other known cases--confirmed or suspected--among persons with hemophilia. DHF personnel telephoned all HTCs/physicians who had not responded by August 1, 1986.

A total of 240 HTCs/physicians and 34 NHF chapters were sent letters, and written responses were received from 61 (25%) HTCs/physicians. Information was obtained by telephone from 209 of the 213 addressees who had not responded; four NHF chapters could not be reached. In addition, DHF personnel contacted the state health departments of three states that had no reported cases and no HTCs or physicians listed in the NHF directory. From these efforts, eight previously unreported cases of AIDS among persons with hemophilia were identified. Two patients were from California (diagnosis of AIDS 12/84 and 7/85); two were from Oregon (diagnosis of AIDS 3/86 and 7/86); and one each from Colorado (diagnosis of AIDS 3/85), Missouri (5/85), New York (4/85), and Virginia (1/86). In four instances, the physicians assumed that the cases had been reported to the appropriate state health departments. In the other instances, two cases involved physicians who did not realize their legal responsibility to report cases of AIDS to the state; one case involved a postmortem diagnosis of opportunistic infection, of which the physician had been unaware; and one case involved an acquired inhibitor to factor VIII, which the physician did not realize constituted a case of hemophilia-associated AIDS. Reported by National Hemophilia Foundation and associated Hemophilia Treatment Centers; Div of Host Factors, Center for Infectious Diseases, CDC.

Editorial Note

Editorial Note: National surveillance for AIDS cases among persons with hemophilia is maintained through the receipt of standard AIDS case report forms submitted by the state health departments to CDC and through reports (without names) sent directly to DHF by physicians and nurses who care for patients with hemophilia. In the latter case, information is immediately shared with the state health department. The eight unreported cases identified in the CDC-NHF survey represent approximately 3% of all reported hemophilia-associated AIDS cases in the United States. This approximates the percentage of such cases that were reclassified according to the case definition for AIDS revised in 1985 (4).

In interpreting the findings of this survey, it should be noted that approximately 50%-60% of persons with hemophilia in the United States receive care through HTCs or hematologists (CDC data, unpublished). However, this selection bias probably does not significantly distort the results of the survey, because hemophiliacs at greatest risk for contracting AIDS, i.e., those who require extensive concentrated clotting-factor replacement (5), are most likely to be followed by these health care providers. The survey could not determine willingness/unwillingness to perform confirmatory diagnostic procedures such as esophagoscopy or lung biopsy in the hemophiliac population. Conversations with HTC personnel and physicians, however, suggest that confirmatory procedures are usually done. Finally, this approach to validation of the surveillance system assumes that physicians who do not initially choose to report AIDS cases (e.g., for reasons of confidentiality) would do so when contacted personally. This may not be the case. Nevertheless, the survey described here and other studies (6,7) suggest that surveillance of AIDS (as currently defined)--particularly of hemophiliacs--is relatively complete.


  1. US Department of Health, Education, and Welfare. Study to evaluate the supply-demand relationships for AHF and PTC through 1980. Washington, DC: US Department of Health, Education, and Welfare, 1977; publication no. 77-1274.

  2. Jason J, Holman RC, Kennedy MS, Evatt BL. Longitudinal assessment of hemophiliacs exposed to HTLV-III/LAV. In: Program and abstracts of the 26th Interscience Conference on Antimicrobial Agents and Chemotherapy. New Orleans, Louisiana;1986:97.

  3. National Hemophilia Foundation. Directory of hemophilia treatment facilities, 1986 edition. New York: National Hemophilia Foundation, 1986.

  4. CDC. Revision of the case definition of acquired immunodeficiency syndrome for national reporting--United States. MMWR 1985;34:373-5.

  5. Gjerset GF, McGrady G, Counts RB, Martin PJ, Jason J, Kennedy S, Evatt B, Hansen JA. Lymphadenopathy-associated virus antibodies and T cells in hemophiliacs treated with cryoprecipitate or concentrate. Blood 1985;66:718-20.

  6. Chamberland ME, Allen JR, Monroe JM, et al. Acquired immunodeficiency syndrome in New York City. Evaluation of an active surveillance system. JAMA 1985;254:383-7.

  7. Hardy AM, Starcher ET, Druker J, Krystal AR, Day J, Kelly C. Using death certificates to determine the level of AIDS case reporting. Second International Conference on AIDS. Paris, France, June 23-25, 1986. *The AIDS virus has been variously termed human T-lymphotropic virus type III (HTLV-III/LAV), lymphadenopathy-associated virus (LAV), AIDS-associated retrovirus (ARV), or human immunodeficiency virus (HIV). The designation "human immunodeficiency virus" (HIV) has been accepted by a subcommittee of the International Committee for the Taxonomy of Viruses as the appropriate name for the retrovirus that has been implicated as the causative agent of AIDS (Science 1986;232:697).

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