Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: 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)

We have studied two patients, one with red cell aplasia and the other with neutropenia. Both showed lymphocytosis. In both cases, 90-100% of E rosetting cells were T cells as defined by the monoclonal antibodies UCHT1 and OKT3. The majority of these cells also carried the OKT8 suppressor/cytotoxic marker and were HLA-DR- and Fc gamma R-positive. In spite of the similarity of this phenotype to that reported for suppressor cells, these cells failed to suppress pokeweed mitogen-induced polyclonal Ig synthesis. Cells from both patients also failed to respond significantly to Con A and PHA. They were, however, unable to suppress the Con A responses of normal donors although cells from one patient were able to suppress completely a normal PHA response. These results demonstrate the existence of a genuine subset of T cells with Fc gamma receptors but suggest that not all such cells have typical suppressor function.
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PMID:Unusual phenotype and function of an expanded subpopulation of T cells in patients with haemopoietic disorders. 702 93

The neutrophil-specific antigen NC1 is defined by an antibody in the serum of a mother who gave birth to a child with alloimmune neonatal neutropenia. NC1 has been reported to be associated with the neutrophil-specific antigen NA2, but the precise relation of NC1 and NA2 remained unclear. Therefore, we investigated the serum using the antigen capture assay MAIGA and the granulocyte (GIFT) and lymphocyte (LIFT) immunofluorescence tests. In GIFT, no NA association was observed. In LIFT, serum antibodies bound preferably to lymphocytes with the HLA antigens HLA-B7 and cross-reacting antigens. In MAIGA, an antibody specific for the NA2 variant of the granulocyte Fc gamma-receptor III was observed. The NA2 specificity was confirmed by testing granulocytes from 40 further different donors. This indicates that the NC1 and NA2 antigens are identical. A positive GIFT result but a negative one in LIFT using cells of an NA2-negative typed individual suggest the presence of an additional, non-NA2-specific granulocyte antibody.
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PMID:Evidence that the granulocyte-specific antigen NC1 is identical with NA2. 753 89

Six antineutrophil antibody (ANA)-positive patients with copper deficiency were classified into two groups; those with (group A, n = 3) and those without (group B, n = 3) neutropenia. The percent binding of ANA for normal peripheral neutrophils was similar in both groups (83.5 +/- 7.2 vs. 79.1 +/- 10.5%). The percent binding of sera to cultured promyelocytic leukemia cells (HL-60) was increased in group A (from 3.7 +/- 3.2 to 12.2 +/- 2.3%) but not in group B (from 65.3 +/- 21.7 to 40.7 +/- 6.3%) after stimulation of HL-60 with DMSO. The stimulated HL-60 cells expressed CD 16 and CD 11b antigens. In the presence of monoclonal antibody for CD 16, the titer of ANA was nil in group A and unchanged in group B. Thus, ANA of patients with neutropenia may recognize mainly the CD 16 antigen, the Fc gamma receptor III of neutrophils.
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PMID:Characterization of antineutrophil antibodies in patients with neutropenia associated with nutritional copper deficiency. 861 Apr 76

Agranulocytosis is a well recognized but uncommon complication of procainamide (PA) therapy, whereas a lupus-like syndrome occurs in approximately 20% of patients treated chronically with PA. In order to gain insight into the immunopathogenic relationships among these conditions, we compared the humoral immune abnormalities in these patient groups as well as in asymptomatic PA-treated patients. A relatively uniform profile of IgM but not IgG autoantibody reactivity with a set of chromatin-related antigens was observed in eight elderly men who developed agranulocytosis after treatment with PA. In contrast PA-induced lupus patients had predominant reactivity with [(H2A-H2B)-DNA] in both IgM and IgG classes. Five of eight patients with agranulocytosis had elevated levels of neutrophil-reactive IgG which appeared to be due to immune complexes based on Fc gamma receptor blocking studies. However, 12 of 15 patients with PA-induced lupus, none of whom had neutropenia, had similar levels of neutrophil-reactive IgG, suggesting that this reactivity was not causally related to agranulocytosis. Agranulocytosis developed after less than 3 months treatment with PA in six of eight patients. This time course was similar to that seen in 77 PA-induced agranulocytosis patients reported in the literature plus 127 patients reported to the U.S. Food and Drug Administration in whom 90% developed agranulocytosis within 3 months of starting PA. In contrast, the mode duration of treatment with PA before lupus-like symptoms develop is 10-12 months. These findings, together with the different profiles of autoantibodies and clinical presentations, suggest that agranulocytosis arises from a different mechanism than that underlying PA-induced lupus.
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PMID:Procainamide-induced agranulocytosis differs serologically and clinically from procainamide-induced lupus. 862 53

An 8-year-old boy had been suffering from chronic autoimmune neutropenia for more than 5 years. The neutropenia proved to be resistant to high-dose steroids and intravenous (either low-or high-dose) immunoglobulin (Ig) therapy. The chronic autoimmune thrombocytopenia and recurrent phases of autoimmune haemolytic anaemia did, however, respond to high-dose prednisone. Other signs of immune dysregulation in this patient consisted of insulin-dependent diabetes mellitus type I (IDDM) and an acquired hypogammaglobulinaemia, most compatible with common variable immunodeficiency (CVI). Prior to rhG-CSF therapy the child had suffered for more than 2 years from recurrent life-threatening bacterial infections. Anti-neutrophil autoantibodies had pan-Fc gamma RIII (CD116, NA1/NA2) specificity. The neutropenia as well as the antineutrophil autoantibodies disappeared when subcutaneous rhG-CSF therapy was started. Upon tapering rhG-CSF, anti-Fc gamma RIII antibodies reappeared together with an absolute neutropenia. Renewed administration resulted again in the normalization of symptoms. Soluble Fc gamma RIII (sFc gamma RIII) antigen levels in plasma increased dramatically during rhG-CSF treatment. These high levels of sFc gamma RIII together with increased numbers as well as decreased apoptotic reactions of neutrophils apparently result in adsorption of the autoantibodies in vivo, contributing to the normalization of autoimmune-mediated neutropenia upon rhG-CSF treatment. Long-term administration of rhG-CSF represents as alternative in the treatment of autoimmune neutropenia.
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PMID:The use of rhG-CSF in chronic autoimmune neutropenia: reversal of autoimmune phenomena, a case history. 907 39

The purpose of this study was to compare two different in vitro culture conditions for the preservation of human granulocytes. These cells could be used in patients with severe neutropenia following cytotoxic chemotherapy if the functional capacity was retained, and autologous transfusions of granulocytes would circumvent the risk of alloimmunization. Granulocytes were obtained from the peripheral blood of healthy donors and patients with hematologic malignancies who received cytotoxic chemotherapy supported by recombinant human granulocyte colony-stimulating factor (R-metHuG-CSF, 300 micrograms/day, s.c.). Granulocytes were either cultured for 72 h at 4 degrees C in the presence of 100 ng/ml G-CSF or cryopreserved at -196 degrees C. The viability, surface antigen expression, and function of the granulocytes were assessed. Since effective microbial killing involves the attachment of granulocytes to blood vessel walls, transmigration into tissues, chemotaxis, and phagocytosis, the surface expression of the adhesion molecules LFA-1 (CD11a/CD18) and gp 150,95 (CD11c/CD18) was measured. In addition, the IgG receptors Fc gamma RI (CD64), Fc gamma RII (CD32), and Fc gamma RIII (CD16), as well as the complement receptor CR3 (CD11b/CD18), were assessed. Dynamic superoxide anion release served as a measure of the metabolic pathway of the oxidative burst after f-Met-Leu-Phe (fMLP) and phorbol-12-myristate-13-acetate (PMA) stimulation. Substantial differences in the preservation of granulocyte integrity and function were observed between the two storage conditions. Cryopreservation abolished reactivity to extracellular stimuli and severely affected the cell phenotype. On the other hand, functional activity could be maintained for up to 72 h when in vivo primed granulocytes of patients were incubated at 4 degrees C in the presence of G-CSF. This storage modality may permit the use of granulocyte autotransfusion to reduce the risk of neutropenic fever.
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PMID:Granulocytes harvested following G-CSF-enhanced leukocyte recovery retain their functional capacity during in vitro culture for 72 hours. 887 10

Polymorphic structures of the neutrophil Fc gamma receptor IIIb (Fc gamma RIIIb) result in alloantibody formation that causes alloimmune neonatal neutropenia and transfusion reactions. Alloantigens located on Fc gamma RIIIb include the antigens NA1 and NA2. In four cases of alloimmune neonatal neutropenia, granulocyte-specific alloantibodies directed against a thus far unknown antigen were detected by granulocyte agglutination and immunofluorescence tests in the maternal sera. By the use of the monoclonal antibody-specific immobilization of granulocyte antigens (MAIGA) assay, the new antigen, termed SH, was located on the Fc gamma RIIIb. Nucleotide sequence analysis of the Fc gamma RIIIb coding region from a SH(+) individual showed a single-base C-->A mutation at position 266, which results in an Ala78Asp amino acid substitution. A family study confirmed that this nucleotide difference is inherited, and corresponds to the SH phenotype. Serologic typing of 309 randomly selected individuals showed an antigen frequency of 5% in the white population. The same frequency was found by genotyping, for which a technique based on polymerase chain reaction (PCR) using sequence-specific primers (PCR-SSP) was developed. Typing of all SH(+) individuals for NA1 and NA2, and PCR-restriction fragment length polymorphism analysis of the NA-specific PCR products from five SH(+) individuals using the SH-specific endonuclease SfaN 1 showed that SH antigen is very probably the result of an additional mutational event in the NA2 form of the Fc gamma RIIIB gene. Immunochemical studies also demonstrated that the SH determinants reside on the 65- to 80-kD NA2 isoform of the Fc gamma RIIIb. Our findings show the existence of an additional polymorphism of the Fc gamma RIIIb, which can result in alloantibody formation causing alloimmune neonatal neutropenia.
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PMID:Characterization of a new alloantigen (SH) on the human neutrophil Fc gamma receptor IIIb. 902 35

Several advances have been made in understanding the polymorphisms of the neutrophil Fc-gamma-receptor IIIb (Fc gamma RIIIb). In one recent study, 21 individuals whose neutrophils lack Fc gamma RIIIb were found to be missing the entire Fc gamma RIIIB and Fc gamma RIIC genes. Another polymorphism of Fc gamma RIIIb, SH, has been characterized. New methods to determine the genotype of Fc gamma RIIIB for NA1, NA2, and SH using leukocyte genomic DNA have been described. A new monoclonal antibody to neutrophil-specific antigen NB1 was produced. Advances have been made in understanding alloimmunization in granulocyte transfusion recipients and the treatment of autoimmune neutropenia with granulocyte colony-stimulating factor (G-CSF). Granulocyte transfusion recipients were found to be alloimmunized both to neutrophil-specific and HLA antigens, suggesting that the transfusion of these patients with granulocytes matched only for HLA antigens will not be effective. A case report suggests that the beneficial effects of G-CSF on patients with autoimmune neutropenia is due in part to G-CSF's action of increasing plasma levels of soluble Fc gamma RIIIb.
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PMID:Neutrophil antibodies. 935 5

Receptors for the constant part of IgG (Fc gamma R) are implicated in the pathogenesis of a number of diseases. Children from mothers with an Fc gamma RIIIb deficiency may suffer from neonatal neutropenia due to an alloimmune reaction. Interindividual differences (polymorphisms) for a number of Fc gamma R represent risk factors for several infectious and autoimmune diseases. Immunotherapeutic use of several monoclonal antibody subclasses is affected by Fc gamma R polymorphisms. Fc gamma R can be used for cellular entrance by lymphotropic viruses (such as HIV) and appear to be involved in the pathogenesis of fulminant Dengue virus infections.
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PMID:[Immunology in clinical practice. XI. IgG receptors: the role of polymorphism in autoimmune and infectious diseases]. 956 39

Three children (girls) suffered from neutropenia mediated by anti-neutrophil IgG-Fc receptor type III (Fc gamma RIII) antibodies. The first patient (newborn) had asymptomatic and transient neutropenia caused by maternal Fc gamma RIII iso-antibodies. The second patient (6 months), whose neutropenia was diagnosed as a 'benign neutropenia of childhood' caused by transient anti-NAI autoantibodies, suffered from mild bacterial infections. The third patient (12 years) suffered from serious infections. The anti-Fc gamma RIII autoantibodies showed neither anti-NA1 nor anti-NA2 specificity. She also developed autoimmune thyroiditis (Graves' disease). Both the duration of the neutropenia and the seriousness of the bacterial infection were variable in our patient group. The first two patients both made spontaneous recoveries, while the third patient depended ultimately on granulocyte-colony stimulating factor (G-CSF).
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PMID:[Neutropenia due to antibodies against Type III Fc receptors on neutrophil granulocytes: 3 children with different clinical courses]. 956 43


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