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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

To examine the role of antihemophilic factor (factor VIII) preparations in the pathogenesis of subclinical immunodeficiency in hemophilia, we tested the in vitro effects of these products on immune function. Both lyophilized antihemophilic factor (LAHF) and cryoprecipitates inhibited lymphocyte proliferation in a dose-dependent fashion. Further studies indicated that LAHF interfered with an early event in proliferation and also that prolonged incubation of human lymphocytes with LAHF resulted in an irreversible inhibition of lymphocyte proliferation without detectable cytotoxic effects. LAHF also inhibited the production of interleukin-2 (IL-2) by human lymphocytes and by Jurkat tumor cells, suggesting that inhibition of IL-2 production was not mediated through effects on interleukin-1. Gel filtration of LAHF revealed two peaks of inhibitory activity; one with mol wt greater than 2 X 10(6) comigrated with factor VIII coagulant activity and antigen, whereas another with mol wt approximately 6 X 10(5) was devoid of factor VIII activity and antigen. Further study will ascertain whether administration of factor VIII-containing preparations contributes to the subclinical immunodeficiency seen in patients with hemophilia or serves as a cofactor in the development of clinical immunodeficiency after exposure to the retrovirus human T-lymphotropic virus type III.
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PMID:Antihemophilic factor [factor VIII] preparations inhibit lymphocyte proliferation and production of interleukin-2. 308 91

The detection of human immunodeficiency virus (HIV) in hemophiliacs treated with factor VIII has prompted the Government of China to institute a national surveillance program. An earlier survey of 310 healthy individuals and leukemia patients from 8 provinces failed to yield any sera positive for antibodies to HIV. However, given the documented high seroprevalence rate among hemophiliacs, the sera of 28 Chinese hemophiliacs and 1 acquired immunodeficiency syndrome (AIDS) patient were analyzed by enzyme-linked immunosorbent assay (ELISA). Antibodies to HIV were detected in the AIDS patient and in 4 of the hemophiliacs. All 4 of the seropositive cases were from a group of 18 hemophiliacs who had received factor VIII produced by the US-based Armour Company. This suggests that HIV infection was transmitted to China from the US via blood products at a frequency of 22%. At the time of the study, all 4 seropositive hemophiliacs were asymptomatic.
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PMID:Detection of antibody to LAV/HTLV-III in sera from hemophiliacs in China. 310 38

The routes of transmission of human immunodeficiency virus (HIV) can be divided into two categories, 1. sexual transmission (male-to-male, male-to-female and female-to-male) and 2. parenteral transmission by blood or blood products. Among 488 Japanese hemophiliacs, 165 (33.8%) were seropositive and were infected by the injection of factor VIII or factor IX manufactured from American sources. The rates of infection among hemophiliacs were 21.8% (19/87) and 36.8% (43/117), respectively for 1984 and 1985. Sera of 10,272 volunteer blood donors were all negative for anti-HIV. There have been 16 confirmed AIDS cases in Japan consisting of 8 male homosexuals and 8 hemophiliacs, so it can be concluded HIV infections have exclusively occurred among male homosexuals and hemophiliacs. But there are chances to transmit HIV by blood transfusion if the screening of whole blood units is not implemented in the near future.
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PMID:Prevalence and transmission of human immunodeficiency virus in Japan. 310 39

Four patients with hemophilia A have undergone liver transplantation in our institution, three successfully. The first was a 21-year-old man with chronic active hepatitis (CAH) in whom the effects of previous abdominal operations prevented the satisfactory technical insertion of the new liver. He died intraoperatively. The second patient was a 15-year-old boy with CAH who began to synthesize factor VIII coagulant activity (F VIII:C) within 18 hours of successful liver transplantation and has continued to do so for almost 2 years (F VIII:C range 0.89 to 3.20 U/mL). The first 2 months of his postoperative course were complicated by infections, but since that time he has done well and has returned to school. The third patient was a 48-year-old man with portal fibrosis and severe ascites. He synthesized F VIII:C (range 0.96 to 1.50 U/mL) within six hours after reestablishment of circulation through the new liver. His postoperative course was complicated by numerous infections, and he died with sepsis and an acquired immunodeficiency-like syndrome 4 months after transplantation. The fourth patient was a 47-year-old mild hemophiliac with CAH who produced adequate factor VIII:C levels following transplantation (range 0.79 to 2.80 U/mL). These patients demonstrate that liver transplantation in hemophiliacs with end-stage liver disease may be lifesaving and results in correction of the F VIII:C deficiency and associated hemorrhagic tendency.
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PMID:Liver transplantation in hemophilia A. 1947 Apr 40

Because there have been reports that factor IX concentrate is less immunosuppressive and therefore factor IX users have less immunologic aberrations, we have studied a group of 22 patients with hemophilia B and six patients with factor VIII deficiency and high titer inhibitors with respect to lymphocyte numbers and function, human immunodeficiency virus (HIV) serology, and factor usage. This group was compared to 111 patients with hemophilia A and a group of 28 healthy male volunteer controls. When the study began in 1983, the majority of patients with hemophilia B and with higher titer factor VIII inhibitors were seronegative, 77% and 83% respectively, as compared to only 30% of patients with hemophilia A. At that time the factor IX users also had milder immune aberrations than the hemophilia A group. However, with time and increasing clotting factor concentrate usage, seroconversion and more striking abnormalities in immune function have occurred in the hemophilia B group. In a subgroup of 16 patients with hemophilia B studied twice, the incidence of seropositivity increased from 31% in 1983 to 69% in 1985. We thus conclude that factor IX concentrate in itself is not less immunosuppressive than factor VIII concentrate. Seroconversion in factor IX concentrate users appears to be lagging behind seroconversion in factor VIII concentrate users, perhaps secondary to the lower cumulative dosage of concentrate that patients with hemophilia B utilize.
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PMID:Immunologic aberrations, HIV seropositivity and seroconversion rates in patients with hemophilia B. 310 24

Induction of immune tolerance in patients with severe hemophilia A and inhibitors to factor VIII was attempted by daily infusion of 50 U of factor VIII per kilogram of body weight without adjunctive immunosuppressive drugs. Modest initial anamnestic elevation of inhibitor levels occurred in six patients within the first month of therapy; inhibitor levels then fell sharply. The other six patients had no increase in inhibitor levels while on the study protocol. Inhibitors became undetectable within one to ten months in nine of the patients; they now receive smaller and less frequent infusions of factor VIII to maintain suppression. Inhibitors were not eradicated in three patients, who had the highest baseline and historic inhibitor levels. Ten patients gave consent for human immunodeficiency virus (HIV) testing; three had no antibody to HIV at the outset. Two of these patients seroconverted while on the protocol, one of whom had received only donor-screened, heat-treated factor VIII. Thus, the benefits of inhibitor suppression must be weighed against the risks of HIV seroconversion or transient elevation of inhibitor levels, as well as against the cost of the factor concentrate used.
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PMID:Induction of immune tolerance to factor VIII in hemophiliacs with inhibitors. 311 78

Sequential serum samples from 18 haemophiliac patients exposed simultaneously to human immunodeficiency virus type 1 (HIV 1) in early 1984 were tested retrospectively for serological markers of infection. Assay for total antibodies to HIV established that the time to seroconversion might be as long as 110 days after exposure to contaminated factor VIII; serum samples were also tested by Western blotting, by enzyme linked immunosorbent assay (ELISA) for specific antibodies to envelope and core proteins, and for p24 antigen by two assay systems during the two years after infection. The studies showed that five of the 12 patients for whom serum samples obtained between exposure and seroconversion were available had transient p24 antigenaemia. Although amounts of total antibody to HIV and of antibodies to envelope proteins rose continuously during the two years of the study, amounts of antibody to the core protein were variable and tended to decline in patients who became symptomatic. Two patients had persistent p24 antigenaemia that began four months after seroconversion; these patients remained asymptomatic. One patient who developed the acquired immune deficiency syndrome (AIDS) had transient antigenaemia at the time of seroconversion but failed to show any antigen for the rest of the study; progression to AIDS was accompanied by an increase in antibodies to envelope proteins. Much of the variability in the course of infection with HIV must represent the differences in the susceptibility of the patients to infection.
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PMID:HIV antigen and antibody detection: variable responses to infection in the Edinburgh haemophiliac cohort. 312 20

Five patients with classic hemophilia were found to have primary pulmonary hypertension, a disorder not previously recognized in this population. All patients had had their coagulation disorder treated for 10 years or more with self-administered lyophilized concentrates of factor VIII, and all had antibodies to human immunodeficiency virus (HIV). Primary pulmonary hypertension was confirmed by histologic means at autopsy in one patient and by lung biopsy findings in another. In the other three patients, the findings are in agreement with this diagnosis. No patient had underlying cardiac or pulmonary disease, or clinical or pathologic evidence of collagen-vascular disease, vasculitis, parasitic disorders, hemoglobinopathy, or exposure to anorexigenic agents. Whether the primary pulmonary hypertension was related to treatment with lyophilized factor VIII, or to the presence of antibodies to HIV, or both, is unknown.
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PMID:Primary pulmonary hypertension in patients with classic hemophilia. 280 26

A patient with hemophilia A and transfusion-associated end-stage chronic liver disease underwent orthotopic liver transplantation. He had no requirement for exogenous factor VIII replacement during the 27 mo he survived. Although his hemophilia was cured, he had antibodies to the human immunodeficiency virus; ultimately he died of complications arising from acquired immunodeficiency syndrome. Liver transplantation for cirrhotic hemophiliacs can free them of the need for antihemophilic-factor therapy; however, application of this approach may be limited by the high prevalence of human immunodeficiency virus infection in multitransfused hemophiliacs.
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PMID:Cure of hemophilia A by orthotopic liver transplantation. 313 Nov 78

Cultures of peripheral blood mononuclear cells for human immunodeficiency virus type 1 (HIV-1) and assays for the p24 antigen were performed for a group of 75 unselected hemophiliacs to determine whether patients positive for HIV-1 antibody are actively infected rather than immunized by viral proteins in non-heat-treated factor VIII or IX concentrates. Fifty-six (75%) of the 75 hemophiliacs were antibody positive and 55 (98%) of the 56 with antibodies also had positive cultures. The one culture-negative individual had detectable HIV-1 proviral DNA sequences in three separate samples of peripheral blood mononuclear cell DNA, as detected by a polymerase chain reaction assay. Detection of serum p24 antigen and the time to development of a positive culture were significantly more frequent and shorter, respectively, in symptomatic vs asymptomatic patients. None of the 19 hemophiliacs negative for HIV-1 antibody had positive cultures, detectable p24 serum antigen, or symptoms of HIV-1 infection. Moreover, latent HIV-1 infection was not detected in 16 female sexual partners of hemophiliacs positive for HIV-1 antibody using Western blot testing, assays for p24 antigen, HIV-1 cultures, and polymerase chain reaction assays, despite repeated unprotected sexual exposure. We conclude that antibody-positive hemophiliacs have been actively infected by HIV-1 and that a long period of latent HIV-1 infection prior to overt seroconversion is unlikely.
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PMID:Hemophiliacs with HIV antibody are actively infected. 249 13


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