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)

A method has been developed for the demonstration of increased platelet surface IgG that uses 1 ml of blood regardless of the platelet count. Platelets are gel filtered to remove plasma and contaminating lymphocytes. They are then reacted with fluorescein-conjugated antihuman IgG and analysed by flow cytometry. Percent positive staining cells vary from 10% to 80% of total cells examined. A platelet antibody index is derived from the product of percent positive staining cells X mean fluorescence intensity of positive staining cells. All patients studied with chronic idiopathic thrombocytopenia purpura (ITP) or human immunodeficiency virus-1 (HIV-1)-related thrombocytopenia had increased platelet surface IgG. Twelve acute children and 11 chronic children had indices averaging 3.5- and 8.9-fold greater than 12 normal children, respectively. Five of 12 children with acute ITP had normal platelet IgG. There was no linear correlation between the platelet antibody index and platelet count. Platelets of patients with acute, chronic, or HIV-1-related ITP displayed autofluorescence. In chronic ITP, the percentage of platelets displaying autofluorescence had a significant negative correlation with the platelet count. This technique will be a valuable diagnostic tool in the pediatric population.
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PMID:Micromethod for demonstrating increased platelet surface immunoglobulin G: findings in acute, chronic, and human immunodeficiency virus-1-related immunologic thrombocytopenias. 236 94

Fourteen Rhesus-positive patients with idiopathic thrombocytopenic purpura or thrombocytopenia associated with human immunodeficiency virus (HIV) infection were treated with intravenous Rhesus antibodies. The mean total dose was 6,800 micrograms administered over a 3 to 6 days' period. A significant rise in platelet count was observed in 13 of the 14 Rhesus-positive patients, but not in 3 Rhesus-negative patients used as controls. This rise was usually transient, lasting less than 3 weeks, but it persisted for more than 3 months in 2 cases. Treatment was well tolerated; transient signs of haemolysis appeared in 11 patients who did not require transfusion. Rhesus antibodies are quick-acting, the number of platelets being doubled as early as the second day of treatment. Interaction between antibody-coated red blood cells and macrophages is a possible mode of action and a feasible therapeutic approach in selected patients with autoimmune of HIV-mediated thrombocytopenia.
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PMID:[Treatment of idiopathic thrombocytopenic purpura and thrombocytopenia associated with the HIV virus using anti-Rhesus immunoglobulins]. 252 99

Platelet-directed antibodies and circulating immune complexes (CIC) were removed from plasma of patients with human immunodeficiency virus (HIV) infection and idiopathic thrombocytopenic purpura (ITP) by extracorporeal immunoadsorption using columns of Staphylococcal protein A-silica (Prosorba columns). In addition, stimulation of a broadly cross-reactive anti-F(ab')2 antibody response was observed. These antibodies also appeared to play a role in the additional removal of platelet-directed immunoglobulins (Igs) and CIC from plasma. Removal of these components from plasma was associated with diminishing levels of antibodies and CIC on patient platelets and significant increases in platelet counts. Extracorporeal immunoadsorption of IgG and CIC from plasma is a beneficial new treatment modality for HIV-associated ITP.
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PMID:Reduction in platelet-binding immunoglobulins and improvement in platelet counts in patients with HIV-associated idiopathic thrombocytopenia purpura (ITP) following extracorporeal immunoadsorption of plasma over staphylococcal protein A-silica. 254 Jul 35

The IgG and IgA antibody subclass responses to pneumococcal polysaccharide antigens type 6A and 19F were studied after immunization with a 14-valent vaccine (Pneumovax, MSD), in 53 splenectomized patients (11 Hodgkin's disease, 13 nonHodgkin's lymphoma (NHL), 9 immune haemolytic anaemia or idiopathic thrombocytopenia purpura and 20 posttraumatic splenectomized patients) and 18 non-splenectomized controls. The antibodies were mainly restricted to the IgG2 and IgA2 subclasses. NHL patients had lower pre-vaccination values to the studied antigens and lower antibody response to vaccination than the other patient groups in which the antibody responses did not differ from those of controls. 1 vaccinated NHL patient experienced two episodes of pneumococcal septicaemia, both occurring after chemotherapy which abolished the previously normal IgG2 antibody levels to the pneumococcal antigens. It is concluded that the antibody response to 6A and 19F antigens after pneumococcal vaccination is not reduced in splenectomized patients but is impaired in immunodeficiency states associated with B-cell lymphoma and treatment with cytostatic drugs.
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PMID:Type-specific anti-pneumococcal antibody subclass response to vaccination after splenectomy with special reference to lymphoma patients. 261 13

Human immunodeficiency virus (HIV) infection is associated to different clinical and laboratory hemostatic alterations. The most important is thrombocytopenia, which very often leads to a picture indistinguishable from idiopathic thrombocytopenic purpura with poor clinical relevance but with possible important problems of differential diagnosis. Thrombotic thrombocytopenic purpura is sometimes associated to HIV infections, although only few reports there exist. Finally, the high incidence of lupus anticoagulant in some phases of HIV infection has not a clinical relevance, but is essentially a laboratory finding which is to be known because it can cause a prolonged partial thromboplastin time.
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PMID:Hemostatic alterations during human immunodeficiency virus infection: a review. 267 75

We present measurements of total platelet-associated immunoglobulin G following platelet lysis (PAIgG tot) and surface-restricted IgG (PAIgG ext) on intact, gel-filtered platelets from 36 normal human donors and 9 patients with human immunodeficiency virus (HIV) infection (CDC stages IIb-IV). For this purpose, an indirect micro ELI-SA technique was developed involving competition between PAIgG and solid-phase absorbed IgG for fixation of conjugated anti-human IgG antibody. In normal donors, the mean values of PAIgG tot was 8.3 +/- 7.4 (mean +/- 2 SD) and of PAIgG ext 4.2 +/- 4.4 fg/platelet. In HIV-infected subjects, PAIgG tot was 37.2 +/- 62.8 and PAIgG ext 17.1 +/- 23 fg/platelet. In healthy subjects the comparison of individual levels of PAIgG tot and PAIgG ext revealed a significant correlation (r:0.763; p: 0.003). These results are compared to those which have been reported in the literature. In addition, the major immunopathological mechanisms considered responsible for immune-mediated idiopathic thrombocytopenic purpura are discussed.
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PMID:Relative importance of total versus external platelet-associated IgG. 275 77

The main immunological abnormality described in patients with the acquired immunodeficiency syndrome (AIDS) is the deficiency in cellular immunity. However, there is increasing evidence of a deficiency of specific antibody synthesis in both symptomatic human immunodeficiency virus (HIV)-infected children and in some HIV-infected adults who are symptomatic or who suffer from AIDS. In uncontrolled studies as well as those using historical controls, iv immunoglobulin (IVIG) has been shown to benefit HIV-infected children with recurrent infection when used in doses similar to those needed to treat patients with primary hypogammaglobulinaemia. Preliminary data in adults show that IVIG therapy reduces bacterial infection but the optimal treatment schedules remain to be defined. In addition, high-dose IVIG treatment (1-2 g kg-1) produces increased platelet counts in patients with life-threatening bleeding due to idiopathic thrombocytopenic purpura associated with HIV infection. Unlike other therapies, IVIG has the advantage of lacking immunosuppressive and other serious adverse effects. In HIV-infected patients, IgG therapy may limit the antigenic stimulation caused by intercurrent infection, and studies of asymptomatic patients are needed in order to investigate whether this might slow the progression to AIDS. Neutralizing antibody against HIV has been demonstrated in HIV-infected patients. Although the significance of this finding is not clear, a specific antibody preparation against HIV may be of value either to prevent HIV infection after initial exposure to the virus or to slow the progression of HIV-related disease. Major difficulties will be encountered in producing a specific, effective, neutralizing anti-HIV immunoglobulin preparation, since it is not known which HIV antigens elicit protective immunity.
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PMID:The treatment of human immunodeficiency virus infected patients with intravenous immunoglobulin. 290 28

The protective effect of passively administered immunoglobulin has been known for many years. Although its accepted use over the last 40 years has been in the prevention and treatment of infection its widest and, arguably, most important application has been in the prevention of Rhesus haemolytic disease of the newborn. However, the development over the last decade of a number of safe and effective immunoglobulin preparations for intravenous use has allowed its more widespread utilisation in clinically appropriate settings. It is now widely accepted that intravenous immunoglobulin (IVIgG) is indicated, on a repeat basis, as treatment of choice as replacement therapy for patients with primary antibody deficiency. It may also be effective, in the non-immune deficient patient, in the management of certain bacterial as well as viral infections. This is particularly the case in the pre-term neonate but may have an application in the infected intensive care patient. The benefit in these patient groups has led to a re-evaluation of the use of immunoglobulin replacement therapy in the severe secondary antibody deficiency states associated with such haematological conditions as multiple myeloma and chronic lymphocytic leukaemia. These are currently the subject of randomised, controlled studies. The observation that the use of IV IgG may be associated with effects other than passive transfer of antibody were first made by Imbach in Switzerland. While treating children with immunodeficiency he noticed that two with a coincident immune thrombocytopenia had a rapid platelet increment in close association with the immunoglobulin infusion. He subsequently confirmed that this was a rapid and predictable form of therapy in childhood idiopathic thrombocytopenic purpura (ITP) and that in some children it appeared to affect the natural history of the disease. Since these initial observations the response has been confirmed in both children and adults and extended to other immune-based haematological disorders.
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PMID:Clinical use of intravenous immunoglobulin in blood disorders. 305 61

Immune serum globulin has been available for approximately 40 years. Although this substance represented a major advance in the treatment of patients with agammaglobulinemia and hypogammaglobulinemia, it has a number of major limitations that restrict its clinical utility. These include the need for intramuscular administration, pain at the site of injection, loss of immunoglobulin G (IgG) extravascularly, limitations on the degree to which serum IgG can be increased, incomplete and delayed onset of absorption, and limitations on the volume of material administered. Intravenous forms of gamma globulin do not have these limitations and, therefore, have been preferred for therapeutic use. While studying the physical chemistry of IgG in solution, it was observed that lowering the pH to the range of 4.0 to 4.5 markedly enhanced its monomer content and stability, obviating the need for any chemical modification, enzymatic treatment, or lyophilization. A new IgG preparation suitable for intravenous administration, IGIV, pH 4.25, has been developed and subjected to extensive clinical testing. It is licensed in the United States (Gamimune N) for replacement therapy of IgG in immunodeficiency syndromes and for the treatment of idiopathic thrombocytopenic purpura. The biochemistry and safety of IGIV, pH 4.25, are reviewed.
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PMID:Overview of the biochemistry and safety of a new native intravenous gamma globulin, IGIV, pH 4.25. 311 8

Pathogenesis of HIV infection and expression of retroviral proteins are gradually being elucidated. Antibody to HIV is a marker of past or present viral infection. The virus can be isolated from cultured lymphocytes of seropositive but not seronegative patients. Sero-epidemiological studies show that the majority of infected patients are asymptomatic carriers without biological sign of immune depression. Some then show immune abnormalities such as a decrease of CD4 cells in the blood; some patients present with lymphadenopathies or signs of AIDS-related complexes. Frank AIDS is a late stage of the disease. Some cofactors increase the immunodeficiency and then accelerate the passage from asymptomatic carrier to persistent generalized lymphadenopathies or AIDS by spreading the virus into target cells, susceptible T4 cells, bone marrow precursors, or brain. These AIDS patients then present with opportunistic infections and/or malignancies like Kaposi's sarcoma, lymphoma, and/or brain diseases (dementia or encephalitis).
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PMID:HIV target cells: effect of their infection by HIV on the pathogenesis of AIDS. 326 Sep 82


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