Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0021051 (immunodeficiency)
 71,517 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The Transfusion Safety Study monitored susceptible persons for human immunodeficiency virus type 1 (HIV-1) infections transmitted by plasma products and blood components. Through December, 1988, 6 subjects without antibody to HIV-1 (anti-HIV-1) became seropositive after receiving dry-heated factor VIII concentrate. The preparations implicated in 3 cases were derived entirely from anti-HIV-1-screened donors. In all instances, HIV-1 infection could be explained by concentrates heated at 60 degrees C for 24-30 h. Limiting consideration to concentrates and components administered after study entry showed that 4 of the seroconversions occurred among 122 subjects given 10 million units of factor VIII concentrates. No seroconversions occurred among 84 subjects given 5 million units of factor IX concentrates, or 83 who received components from over 26,000 unpaid donations. Serologic surveillance of anti-HIV-1-negative subjects provides important information, and should be routine in the management of persons receiving clotting factor concentrates.
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PMID:Transmission of human immunodeficiency virus type 1 by dry-heated clotting factor concentrates. Transfusion Safety Study Group. 212 51

Porcine or bovine factor VIII concentrates (FVIII:C) have been used during the past 3 decades to control bleeding in patients who have developed antibodies to human factor VIII. Since current preparations of animal FVIII:C are not known to transmit infectious agents such as hepatitis or human immunodeficiency virus, they are of potential therapeutic interest. A purified porcine FVIII:C (Hyate:C) is now widely used as an alternative to human FVIII:C in patients with inhibitor. Unlike earlier preparations of porcine FVIII:C, thrombocytopaenia is rare with the current preparation. Nonetheless, it causes the aggregation of human platelets in vitro. Our aim was to identify precisely the plasma factor which induces platelet aggregation. The effects of commercial porcine FVIII:C, porcine fibrinogen, porcine fibronectin and the corresponding preparations from human origin on platelet aggregation were studied. Platelet aggregation was quantified by measuring the fall in single platelet count in human whole blood. Of these preparations, only porcine FVIII:C (0.1-1 U/ml) and porcine fibrinogen (80-600 micrograms/ml) induced a fall in single platelet count of up to 85% due to aggregation. The extent of aggregation was directly proportional to the amount (0.007-0.1 U/ml test aliquot) of residual von Willebrand factor antigen (vWf:Ag) in the preparations. A monoclonal antibody to vWf:Ag inhibited the aggregation. We believe that the aggregation of human platelets induced in vitro by porcine FVIII:C is mediated by vWf:Ag which also may be responsible for thrombocytopaenia reported following administration of porcine FVIII:C in vivo.
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PMID:Further evidence that the residual vWf:Ag in porcine FVIII:C induces human platelet aggregation. 212 38

The treatment of hemophilia A patients has progressed in the last 30 years. The development of more pure factor VIII concentrates has been encouraged by the appearance of the human immunodeficiency virus in blood supply. Monoclate was the first essentially pure form of factor VIII available for hemophilia therapy. The liquid pasteurized form of Monoclate represents a new standard for therapy that attempts to eliminate a broad spectrum of viral content in factor VIII preparations.
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PMID:Preclinical characterization of a new pasteurized monoclonal antibody purified factor VIIIC. 212 54

The prevalence of antibody to human parvovirus B19 was determined in 86 children with congenital bleeding disorders. Forty-seven of 53 boys (89%) receiving non-heat-treated factor VIII or prothrombin complex concentrates were anti-B19 IgG positive compared with 38% of their age-matched controls and 48% of children treated with cryoprecipitate. Acute B19 virus infection occurred in 2 boys 3-4 weeks after they had received the same batch of commercial factor VIII concentrate. Of 11 susceptible children who had only received heat-treated National Health Service factor VIII concentrate (8Y), 1 acquired anti-B19 IgG. This suggests that 8Y heat-treated concentrate has a much reduced risk of transmitting B19 virus and, by implication, other less heat-stable viruses such as human immunodeficiency virus.
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PMID:Transmission of human parvovirus B19 by coagulation factor concentrates. 216 Jan 47

Conventional clotting factor concentrates have, until recently, been "of intermediate purity," containing less than 1% of the coagulation factor, and greater than 99% extraneous plasma proteins such as fibrinogen, fibronectin, gamma globulins, and traces of many others. We report here the results of a new factor VIII concentrate that is purified from human plasma using a mouse monoclonal antibody to factor VIII:vWF in an affinity chromatography system. The resultant concentrate has an activity of between 3,000 and 5,000 U/mg protein before albumin is added as a stabilizer. Seven patients with severe hemophilia A and no inhibitor who were positive for antibody to human immunodeficiency virus (HIV) have been treated solely with this concentrate for over 24 months. Factor usage in these patients has ranged from 611 U/kg/yr to 2,022 U/kg/yr. These patients have infused approximately once per week on the average, most often for joint hemorrhages. The efficacy of the concentrate is excellent. No allergic reactions have occurred and no factor VIII antibodies have developed. In these seven patients mean CD4 counts stabilized (856 +/- 619 at screen v 778 +/- 686 at 24 months) and there was reversal of skin test anergy. In a comparison group on conventional intermediate purity concentrate chosen retrospectively decreases in mean CD4 cell counts similarly did not occur. However, the number of the comparison patients who were anergic increased over the course of the study. These observations indicate the possibility that more highly purified concentrates may stabilize immune function in HIV seropositive patients.
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PMID:Factor VIII:C concentrate purified from plasma using monoclonal antibodies: human studies. 249 75

The spontaneous disappearance of the inhibitor to factor VIII (FVIII) was observed in two human immunodeficiency virus (HIV)-infected men with hemophilia A. Both men had end-stage HIV infection, one with acquired immune deficiency syndrome (AIDS) and one with severe AIDS-related complex (ARC). Loss of the inhibitor was associated with a fall in T4 helper lymphocytes to less than 100 per mm3 in both patients. Subsequent spontaneous and traumatic hemorrhages were treated successfully with standard doses of FVIII concentrate, resulting in adequate FVIII:C levels and good hemostasis. The mechanism by which the anti-FVIII inhibitor disappears is not known, but it is likely to be related to a quantitative decline in T4 cell number.
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PMID:Disappearance of inhibitor to factor VIII in HIV-infected hemophiliacs with progression to AIDS or severe ARC. 249 58

The prevalence of lupus anticoagulant, using the dilute Russell's viper venom time (DRVT), was determined in 22 patients with mild to severe haemophilia A to see if there was any association with the presence of viral disease. Twelve haemophiliacs (58%) were lupus anticoagulant positive, with a mean patient:control ratio of 1.24 (range 1.15-1.52, normal range 0.84-1.06 which partially corrected with lysed, washed platelets). Nine of these patients were IgG or IgM, or both, anticardiolipin antibody positive and nine were human immunodeficiency virus (HIV) antibody positive, but associations between lupus anticoagulant, anticardiolipin antibodies, and HIV antibody positivity were not significant. Mixing studies of normal plasma and immune depleted factor VIII deficient plasma showed that the DRVT ratio increased when the factor VIII concentration fell below 0.15 IU/ml. There was no significant association between plasma factor VIII concentration and positive DRVT results in haemophiliacs. The addition of porcine factor VIII concentrate produced no correction in eight of the 12 with DRVTs indicative of lupus anticoagulant, suggesting that these were prolonged by antiphospholipid activity. It is concluded that the presence of lupus anticoagulant and anticardiolipin antibodies in haemophiliacs may represent an antiphospholipid response to viraemic challenge, not only to HIV but also to other viral antigens, and that a very low factor VIII concentration may produce a false positive DRVT result.
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PMID:Lupus anticoagulant, anticardiolipin antibodies, and human immunodeficiency virus in haemophilia. 250 Apr 59

The development of factor VIII concentrates has greatly facilitated hemophilia care and has made the home care of hemophilia possible. However, factor VIII concentrate that has been produced using traditional methods contains large amounts of foreign proteins and viruses. This has resulted in the development of immunologic abnormalities in many hemophiliacs and has exposed many of these patients to blood-borne viruses such as the human immunodeficiency virus (HIV) and hepatitis viruses. Factor VIII circulates in plasma in complex with the von Willebrand factor (vWF). Both factor VIII and vWF have been purified and monoclonal antibodies (mAb) have been generated to both of these proteins. When bound to a solid support, these mAb's can be used to isolate selectively the proteins of interest. Recently, two separate procedures have been used in the immunoaffinity purification of factor VIII on a commercial scale. One product (Monoclate) has been prepared using a mAb to the vWF bound to a chromatography column. The other product (Hemophil M) uses immobilized mAb to the factor VIII molecule. Factor VIII concentrate purified using either of these approaches is far more pure than traditional factor VIII concentrates. In addition, the use of both viral purification and viral inactivation procedures has greatly reduced the risk of viral contamination. Early clinical studies have demonstrated that these products are effective in treating bleeding episodes and that the risk of viral infection with HIV or hepatitis viruses is low. Factor VIII concentrate produced using mAb technology appears to be the product of choice in previously untransfused hemophiliacs. Its role in the treatment of patients who have already been infected with HIV is less clear.
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PMID:Immunoaffinity purification of factor VIII. 250 62

Patients with hemophilia A or von Willebrand's disease who are treated with concentrated preparations of human factor VIII made from unscreened pooled plasma are at substantial risk of contracting human immunodeficiency virus (HIV) infection. The purpose of this study was to investigate whether by treating such patients with a pasteurized factor VIII concentrate that had been heated in aqueous solution at 60 degrees C for 10 hours, HIV infection could be avoided. Eleven hemophilia centers in the Federal Republic of Germany and two in Austria identified 155 eligible patients who had been treated exclusively with pasteurized factor VIII concentrate and had not received any other blood products. Between February 1979 and December 1986 they received a total of 15,916,260 IU of pasteurized factor VIII. The United States was the source of 80 percent of the plasma from which the concentrate was made. By September 1988, these 155 patients had been screened for antibody to HIV type 1 (anti-HIV-1) with a total of 657 tests; all were negative. Sixty-seven patients were also tested once for antibody to HIV type 2 (anti-HIV-2); all these tests were negative as well. It appears that pasteurization effectively inactivates HIV, even in plasma that is likely to be highly contaminated with the virus.
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PMID:Absence of anti-human immunodeficiency virus types 1 and 2 seroconversion after the treatment of hemophilia A or von Willebrand's disease with pasteurized factor VIII concentrate. 250 17

To determine laboratory evidence suggesting immunological abnormalities in persons with hemophilia, we evaluated the immunological status of 75 Korean hemophiliacs, seronegative for human immunodeficiency virus (HIV) antibodies, who have been treated only with Korean factor VIII concentrates. From this study, it was shown that Korean hemophiliacs had decreased CD4 levels, increased CD8 levels, and decreased CD4:CD8 ratios. Diminished lymphocyte response to the mitogens, phytohemagglutinin and concanavalin A, and decreased natural killer cell activity were observed in the hemophiliacs. In addition, production of interleukin-II in the hemophiliacs was lower than in the healthy controls. The percentage of B lymphocytes was significantly reduced but the serum levels of immunoglobulin (Ig) G were elevated. However, the serum Ig A and Ig M levels were normal. This study demonstrated a high frequency of immunological abnormalities in HIV antibody negative Korean hemophiliacs treated only with domestic factor VIII concentrates.
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PMID:A comprehensive study of immunological abnormalities in Korean hemophiliacs. 250 31


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