Current Trends Update: Acquired Immunodeficiency Syndrome
(AIDS) among Patients with Hemophilia -- United States
In 1982, six hemophilia A patients who had developed
Pneumocystis
carinii pneumonia (PCP) and other opportunistic infections and who
met
the CDC case definition of AIDS were reported by CDC (1,2). As of
November 30, 1983, physicians and health departments in the United
States have reported a total of 21 AIDS cases among hemophilia
patients--19 among patients with hemophilia A and two among
patients
with hemophilia B. In addition, seven cases from outside the
United
States meeting the CDC definition of AIDS in association with
hemophilia A have been brought to CDC's attention. Of the
hemophilia
cases in the United States, one was diagnosed in 1981; eight, in
1982;
and 12, to date in 1983 (Figure 1). Two patients are known to have
had other risk factors for acquiring AIDS.
To date, no cases of Kaposi's sarcoma have been reported in
association with hemophilia; each patient had an opportunistic
infection suggestive of an underlying cellular immunodeficiency.
PCP
was the most common opportunistic infection in hemophilia patients
with AIDS and has occurred in 20 (95%) of the U.S. patients. Many
of
these patients have had other opportunistic infections, principally
candidiasis, cryptococcosis, toxoplasmosis, and histoplasmosis, or
infections with cytomegalovirus and Mycobacterium
avium-intracellulare. The geographic distribution has included 15
states, with four cases each in the Mid-Atlantic, South Atlantic,
and
East North Central regions, three in the East South Central region,
two each in the New England and West North Central regions, and one
each in the Pacific and Mountain regions. No state was the
residence
for more than two patients.
The National Hemophilia Foundation (NHF) and CDC have conducted
a
mail survey of 116 hemophilia treatment centers (HTCs) designated
by
the NHF in the 48 contiguous states, which estimated the prevalence
of
AIDS-associated diseases from 1978 to 1982 among approximately
6,700
hemophilia patients; a separate review of U.S. deaths reported to
the
National Center for Health Statistics as being hemophilia-related
was
also included in the survey. This survey failed to identify any
diagnoses suggestive of AIDS occurring among hemophilia patients
before the first case diagnosed in September 1981 or any cases
other
than those reported here. In addition to the 21 reported U.S.
hemophilia patients with AIDS, some patients with hemophilia have
been
reported with unexplained, possibly AIDS-associated phenomena that
do
not fit the CDC criteria for an AIDS diagnosis, including
lymphadenopathy syndrome (3), thrombocytopenic purpura (4), and
Burkitt's lymphoma (5).
Reported by S Karp, MS, M Shuman, MD, Moffitt Hospital, University
of
California--San Francisco, S Dritz, MD, City/County Health Dept,
San
Francisco, California; S Marchesi, MD, P McPhedrin, MD, Yale-New
Haven
Hospital, New Haven, Connecticut; AE Pitchenik, MD, University of
Miami, Florida; P Bertagnoll, MPH, Hemophilia of Georgia, Inc.,
Atlanta; D Green, MD, McGaw Medical Center, Northwestern
University, M
Telfer, MD, Michael Reese Hospital, Chicago, G Rifkin, MD, St.
Anthony's Hospital, University of Illinois, Rockford; M Serwint,
MD,
University of Kentucky Medical Center, Louisville; E Mohler, Jr,
MD,
St. Agnes Hospital, Baltimore, Maryland; D Brettler, MD, Worcester
Memorial Hospital, Worcester, Massachusetts; L Rubin, MD,
Children's
Hospital of Long Island Jewish Hillside Medical Center, New Hyde
Park,
A Brownstein, MPH, Executive Secretary, National Hemophilia
Foundation, New York City, New York; E Eyster, MD, Hershey Medical
Center, Hershey, Pennsylvania; SL Green, MD, Riverside Hospital,
Hampton, Virginia; J Craske, MD, Withington Hospital, Manchester,
England; J L'Age-Stehr, Robert Koch Institut, Berlin, West Germany;
Div of Host Factors, AIDS Activity, Div of Viral Diseases, Center
for
Infectious Diseases, Div of Field Svcs, Epidemiology Program
Office,
CDC.
Editorial Note
Editorial Note: Although the etiology of AIDS remains unknown,
epidemiologic evidence suggests an infectious cause (6,7). The
possibility of blood or blood products as vehicles for transmission
of
AIDS to hemophilia patients is supported by the increased risk of
AIDS
in intravenous drug abusers (8) and reports of
transfusion-associated
AIDS cases (9,10). Patients with hemophilia receive transfusions
of
anti-hemophilic factor and plasma factor concentrates prepared from
pools of sera from 2,000 to 20,000 donors. Cryoprecipitate and
plasma
factor preparations are associated with the transmission of several
known viral agents, including cytomegalovirus, hepatitis B virus,
and
the virus(es) of non-A, non-B hepatitis (11). However, at least
nine
U.S. hemophilia-associated AIDS patients also received other blood
products in the 5 years preceding their AIDS diagnoses.
The NHF's Medical and Scientific Advisory Council has issued
specific recommendations for managing hemophilia patients receiving
blood and blood products (12). In addition, the U.S. Public Health
Service has requested that persons at high risk of acquiring AIDS
refrain from donating plasma and/or blood and that an extensive
effort
be undertaken to develop and evaluate the use of laboratory tests
for
screening blood or blood products obtained from individuals in
high-risk groups (13,14). Physicians diagnosing opportunistic
infections or unusual neoplasms in hemophilia patients who have not
received antecedent immunosuppressive therapy are encouraged to
report
these findings to local or state health departments and to CDC.
References
CDC. Pneumocystis carinii pneumonia among patients with
hemophilia A. MMWR 1982;31:365-7.
CDC. Update on acquired immune deficiency syndrome (AIDS)
among
patients with hemophilia A. MMWR 1982;31:644-6, 652.
Gill JC, Menitove JE, Wheeler D, Aster RH, Montgomery RR.
Generalized lymphadenopathy and T cell abnormalities in
hemophilia
J Pediatr 1983;103:18-22.
Ratnoff OD, Menitove JE, Aster RH, Lederman MM. Coincident
classic hemophilia and "idiopathic" thrombocytopenic purpura in
patients under treatment with concentrates of antihemophilic
factor (factor VIII). N Engl J Med 1983;308:439-42.
Gordon EM, Berkowitz RJ, Strandjord SE, Kurczynski EM, Goldberg
JS, Coccia PF. Burkitt lymphoma in a patient with classic
hemophilia receiving factor VIII concentrates. J Pediatr
1983;103:75-7.
Special report. Epidemiologic aspects of the current outbreak
of
Kaposi's sarcoma and opportunistic infections. N Engl J Med
1982;306:248-52.
Jaffe HW, Choi K, Thomas PA, et al. National case-control
study
of Kaposi's sarcoma and Pneumocystis carinii pneumonia in
homosexual men: part 1, epidemiologic results. Ann Intern Med
1983;99:145-51.
Masur H, Michelis MA, Wormser GP, et al. Opportunistic
infection
in previously healthy women: initial manifestations of a
community-acquired cellular immunodeficiency. Ann Intern Med
1982;97:533-9.
Ammann AJ, Wara DW, Dritz S, et al. Acquired immunodeficiency
in
an infant: possible transmission by means of blood products.
Lancet 1983;1:956-8.
Andreani T, le Charpentier Y, Brouet J-C, et al. Acquired
immunodeficiency with intestinal cryptosporidiosis: possible
transmission by Haitian whole blood. Lancet 1983;1:1187-91.
Enck RE, Betts RF, Brown MR, Miller G. Viral serology
(hepatitis
B virus, cytomegalovirus, Epstein-Barr virus) and abnormal
liver
function tests in transfused patients with hereditary
hemorrhagic
diseases. Transfusion 1979;19:32-8.
Medical and Scientific Advisory Council. Recommendations to
prevent AIDS in patients with hemophilia (revised). New York:
National Hemophilia Foundation, October 22, 1983.
CDC. Acquired immune deficiency syndrome (AIDS): precautions
for
clinical and laboratory staffs. MMWR 1982;31:577-80.
CDC. Acquired immunodeficiency syndrome (AIDS): precautions
for
health-care workers and allied professionals. MMWR
1983;32:450-1.
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