Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Fc gamma receptors are a group of three different receptors with several subtypes. They are widely distributed on many cells of the immune system and contribute to the pathogenesis of immune complex- and autoantibody-mediated diseases such as vasculitis, rheumatoid arthritis, idiopathic thrombocytopenic purpura or autoimmune neutropenia. This review focuses on the structure, distribution and function in Fc gamma receptors and their subtypes.
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PMID:[Fc gamma receptors: structure, function, and clinical significance]. 138 31

Thirty-two (18%) of 181 children cared for at our institution who were infected with the human immunodeficiency virus type 1 (HIV-1) were first seen, and HIV was diagnosed, when they were 4 years of age and older. Initial complaints or diagnoses for these children included the following: hematologic disorders (5) (3 idiopathic thrombocytopenic purpura, 1 neutropenia, 1 anemia); recurrent bacterial infections (10); Pneumocystis carinii pneumonia (3); developmental delay (1); skin disorders (2) (1 genital wart, 1 chronic zoster); weight loss (3); malignancy (1); and nephropathy (1). Eight children were referred for evaluation because of maternal HIV-1 infection. The risk factors for HIV-1 infection included maternal/perinatal exposure (22), perinatal blood transfusion (6), blood transfusion during infancy (2), and sexual abuse (2). Ten (31%) of the 32 children have subsequently died. The longest survival from perinatal infection was 12 years. HIV-1 infection in children can result in a prolonged clinical latency and can masquerade as other pathologic conditions. The absence of clinical symptoms in older children at risk for HIV-1 infection should not deter HIV testing.
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PMID:Delayed recognition of human immunodeficiency virus infection in preadolescent children. 140 40

IVIG has been shown to be useful in the treatment of acute and chronic ITP, immune neutropenia, and in some cases of AIHA. The mechanism of action of IVIG is owing to a number of factors, which include Fc blockade, immune modulation of T- and B-cell number and function, alterations in NK activity, and direct effects on autoantibody binding and production via the antiidiotypic antibody network. Current research efforts are directed toward elucidation of these modalities and determination of their relative importance in treating patients with immune-mediated cytopenias.
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PMID:IVIG in the treatment of children with acute and chronic idiopathic thrombocytopenic purpura and the autoimmune cytopenias. 160 24

Forty-three adult patients with idiopathic thrombocytopenic purpura (ITP) were treated by slow intravenous infusions of vinblastine. Nineteen had ITP of recent onset (i.e. of less than 6 months duration) and had contraindication to steroids (3 patients), refractoriness to steroids (6 patients) or to steroids and high dose intravenous immunoglobulins (IVIg, 10 patients). Of the 19 patients, 10 achieved complete response (CR), 2 achieved partial response (PR), 2 had minor response (MR) and the remaining 5 patients had no response (NR). Six of the complete responders remained in CR after 12 to 48 months, whereas all other responders relapsed within 3 months, in spite of maintenance therapy. Twenty-four patients had chronic ITP (i.e. of 6 months duration or more) and had showed no or only transient response to steroids and/or splenectomy, and in many of them, to other therapeutic approaches. Four achieved CR, 4 PR, 6 MR and 10 NR. All but 3 responses were shorter than 3 months, in spite of maintenance therapy. Most responses to slow infusions of vinblastine began after the first infusion. Main side effects included leukopenia in 9 patients (but with absolute neutropenia in only one) and peripheral neuropathy in 2 patients. Interval from diagnosis was the only prognostic factor of response to treatment. We conclude that slow infusions of vinblastine may be a useful approach in ITP of recent onset, when contraindication or refractoriness to steroids and/or IVIg exists. In our experience, this treatment has limited benefit in chronic ITP. In addition, it remains to be demonstrated that slow infusions of vinca alkaloids have any superiority over intravenous bolus injections of the same drugs.
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PMID:Slow infusions of vinblastine in the treatment of adult idiopathic thrombocytopenic purpura: a report on 43 cases. 233 84

The effect of high-dose intravenous gammaglobulin (IVG) therapy with a CLB preparation was studied in 42 patients: 8 patients had acute and 26 patients had chronic idiopathic thrombocytopenic purpura (ITP); 5 patients had thrombocytopenia accompanied by various diseases such as systemic lupus erythematosus, auto-immune haemolytic anaemia and neutropenia; 3 patients had hypoplastic anaemia and 1 patient had neutropenia and rheumatoid arthritis. After treatment, a rise in platelet count occurred in about 75% of the patients with ITP, although there was no sustained response in any of the patients. There was no correlation between the strength of platelet antibodies as detected by the direct immunofluorescence test before infusion and the pattern of response to the infusion. In most cases of ITP, no immune complexes, as measured by Clq-binding assay, were observed. Furthermore, we found no relationship between the amount of Clq-binding activity of patients' sera and the reaction pattern after infusion of IVG. Splenectomy of the patient had no influence on the outcome of IVG therapy.
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PMID:Intravenous gammaglobulin therapy in idiopathic thrombocytopenic purpura. Results with the Netherlands Red Cross immunoglobulin preparation. 241 49

In autoimmune disorders there is usually only one cell-type involved but occasionally there are more. The cell associated-IgG and -C3 in these disorders are sequestered normally in the spleen and removed by phagocytosis in the reticulum endothelial system (RES). Chronic idiopathic thrombocytopenic purpura (ITP) is an autoimmune disease and normally presents only thrombocytopenia. Infrequently, neutropenia is associated. Recently high doses of intravenous gammaglobulin (HDIGG) have been used to block the cell destruction by RES in these diseases. The patient presented with ITP and associated neutropenia and was treated with HDIGG. We studied some neutrophil function, in relation to therapy (chemotaxis, random migration, latex phagocytosis, NBT reduction, superoxide generation), and performed some cytochemical stains (LAP and myeloperoxidase). We also determined the presence of antiplatelet and antineutrophil functions and resulted in an increase in the platelet and neutrophil count.
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PMID:A study of neutrophil function in a case of associated autoimmune neutropenia and thrombocytopenia treated with high doses of intravenous gammaglobulin (HDIGG). 243 24

High-dose immunoglobulin therapy, which is useful for the treatment of idiopathic thrombocytopenic purpura, autoimmune neutropenia, childhood epilepsy, and Kawasaki disease is postulated here to act by immunosuppressing the patients. Evidence provided here shows that the immunoglobulin inhibits phytohemagglutinin (PHA) stimulation, mixed lymphocyte culture (MLC) response, natural killer assay, antibody-dependent cell-mediated cytotoxicity, and cell-mediated lympholysis. These assays were all inhibited by IgG concentrations that exceeded normal levels by 1.5-2 times. It is also shown that Fc fragments were 100-1000 times more effective than intact IgG in inhibiting the PHA and MLC responses. Thus, it is likely that the Fc portion of immunoglobulin functions as an inhibitor of cellular immunity.
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PMID:Evidence for immunosuppression by high-dose gammaglobulin. 243 52

During the last few years the use of intravenous immunoglobulin (IVIG) has attracted increasing interest for the treatment of patients who do not have a classical humoral antibody deficiency syndrome. In certain situations this approach has revolutionized medical management, e.g. in immune thrombocytopenia. In other areas, such as in Kawasaki's syndrome, IVIG therapy have been shown to be highly beneficial in preventing long term disease sequelae by some investigators, but the field remains controversial. Conditions under which IVIG therapy has been shown to be of potential benefit are: (1) intractable childhood epilepsy; (2) autoimmune diseases, e.g. myasthenia gravis, systemic lupus erythematosus, idiopathic thrombocytopenic purpura, idiopathic neutropenia and aplastic anemia; (3) atopic allergy with IgG subclass deficiency including bronchial asthma; (4) in severe infections in combination therapy with antibiotics and as an antipyretic; (5) in Kawasaki's disease; (6) in multiple myeloma and chronic lymphocytic leukemia. Oral and intraventricular administration of IVIG have also been tried, the former for severe diarrhea and the latter to try to rescue the central nervous system from damage by a pathogen. Carefully controlled clinical trials are needed to establish the efficacy of gamma-globulin therapy in these and other conditions.
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PMID:New and controversial uses of intravenous gamma-globulin. 245 9

In 1981, Imbach et al. (Lancet, 1, 1228-1231) reported that infusion of intravenous immunoglobulin (IVIG) would substantially elevate platelet counts in children with acute or chronic idiopathic thrombocytopenic purpura (ITP). Subsequent studies confirmed these findings and extended the effect to adults and to newborns with passive immune thrombocytopenia. Studies in children with acute ITP demonstrated that administration of IVIG was the fastest way to increase a patient's platelet count, and that this agent could be given at doses as high as 1 g/kg/day so that the course of therapy and response to treatment would be more rapid. Reports of effective treatment of patients with autoimmune neutropenia and autoimmune hemolytic anemia by IVIG broadened the scope of its usefulness. In addition, several studies in children and adults with chronic ITP suggested that repeated infusions were a safe and effective way to maintain an adequate platelet count in such patients, and might also gradually lead to lasting improvement. These studies and Imbach's controlled trial in children with acute ITP suggested that IVIG therapy might provide a curative effect in addition to the acute effect. To combine all of these clinical effects with a multitude of in vitro observations to explain the mechanism of action of IVIG is complicated. Fehr et al. (N. Engl. J. Med. 306, 1254-1258, 1982) showed that Fc receptor blockade occurs following administration of IVIG. This effect is clearly demonstrated in vivo by the delayed removal from the vascular space of antibody-coated red blood cells following infusion of IVIG. In addition, many less well-defined effects occur in relation to immunoglobulin production, induction of suppressor cells, antiplatelet antibody levels, and bone marrow platelet production. Studies continue to try to define which effects actually underlie the clinical effects seen, and which are merely test-tube phenomena.
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PMID:The use of intravenous gamma-globulin in idiopathic thrombocytopenic purpura. 247 85

Neutrophil-associated IgG (NAIgG) and neutrophil-binding IgG in sara (NBIgG) of 77 patients with neutropenia suspected to be caused by autoimmune mechanisms (group A) and 31 patients with aplastic anemia or myelodysplastic syndrome (group B) were assayed by flow cytometry. Auto-NBIgG was elevated in 32% of the patients in group A, particularly in about 70% of those with collagen diseases or ITP, but the level was normal in group B. Elevated NAIgG with normal auto-NBIgG levels was found in 27% of the patients in group A and in 64% of the patients in group B. The assay of auto-NBIgG was useful for detection of anti-neutrophil autoantibodies and for the diagnosis of autoimmune neutropenia. In addition, the level of NAIgG may be non-specifically elevated in non-immune neutropenia.
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PMID:[Neutrophil-associated IgG and neutrophil-binding IgG in autoimmune neutropenia]. 258 44


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