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.
References
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.
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.
National Hemophilia Foundation. Directory of hemophilia
treatment
facilities, 1986 edition. New York: National Hemophilia
Foundation, 1986.
CDC. Revision of the case definition of acquired
immunodeficiency
syndrome for national reporting--United States. MMWR
1985;34:373-5.
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.
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.
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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