Acquired Immunodeficiency Syndrome in a Patient with Hemophilia

Abstract

A patient with hemophilia A developed T-cell deficiency characterized by infection with several opportunistic pathogens. Immunologic investigation showed cutaneous anergy, lymphocyte unresponsiveness to mitogens and antigens, an abnormal ratio of T-helper and T-suppressor cells with absolute lymphopenia and elevated IgA. The clinical and immunologic characteristics of this patient fit the recently described syndrome of opportunistic infections or Kaposi's sarcoma in patients with acquired T-cell deficiency; however, this patient does not have any of the associated underlying risk factors such as homosexuality, intravenous drug or amyl nitrite use, or positive serologic tests for syphilis. We conclude that the patient's acquired T-cell deficiency can be explained by exposure to a virus or other transmissible agent during factor VIII transfusions.

Deficiency of the autologous mixed lymphocyte reaction in patients with classic hemophilia treated with commercial factor VIII concentrate. Correlation with T cell subset distribution, antibodies to lymphadenopathy-associated or human T lymphotropic virus, and analysis of the cellular basis of the deficiency.

“…In additional investigations, mitogen and alloantigen responsiveness was found to be similar in patients and healthy controls; in this respect, our study is at some variance with previous reports which have shown reduced responses in hemophilic individuals (3)(4)(5)21 Leu 2a/T8 subset is known to respond well to mitogens and alloantigens in healthy individuals; thus, even for patients with low RT there is no theoretical reason to be bad responders in these assays; second, PBMC have been used in the quoted studies, whereas purified T cells with a defined accessory cell supplement have been employed in the present investigation, an approach that overcomes potential problems with differences regarding the distribution of the populations of immunocompetent cells between individual patients or study populations. Furthermore, the observation of normal IL-2 production in hemophilic patients irrespective of their RT indirectly confirms the finding of normal proliferative responses to mitogens.…”

Functional T lymphocyte immune deficiency in a population of homosexual men who do not exhibit symptoms of acquired immune deficiency syndrome.

“…To date, >70 independent experiments were performed that compared the CTL responses of experimental and control donors. 15 representative experiments are shown in Fig. 1 in which the antiinfluenza responses of one or two The data of the individual experiments shown in Fig.…”

“…All donors exhibited serum antibody activity to influenza A, which indicated that they all had been previously exposed to this virus tionnaire which was concerned with medical history and life style. 15 of the 20 donors responded to the questionnaire. Potentially relevant information taken from the questionnaires is summarized in Table III.…”

“…Subsequently, a number of populations have been identified as being at high risk for AIDS, including homosexual men (4)(5)(6)(7)(8), intravenous drug users (6)(7)(8)(9)(10), Haitians (11)(12), hemophiliac recipients of Factor VIII concentrates (13)(14)(15), recipients of multiple blood transfusions (16,17), sexual contacts ofAIDS patients, and possibly infant children of AIDS patients (18)(19)(20). Despite intensive clinical and research efforts, a number of major problems remain unresolved, including identification of the etiologic agent(s), early diagnosis, and the development of an effective therapeutic protocol.…”

Historical perspective and future direction of coagulation research

“…The availability of factor VIII concentrates has also made possible the home treatment of haemophiliacs, contributing to the improvement of prognosis and quality of life of these patients [103]. However, factor concentrates prepared from a large plasma pool have been contaminated with blood-borne virus including hepatitis B and hepatitis C, as well as HIV, which resulted in the unfortunate spread of HIV infection among haemophiliacs [104]; a tragedy that will never be forgotten. Efforts have been made to improve the safety of plasma-derived factors by adopting measures such as pasteurisation and solvent-detergent that would inactivate virus during production.…”