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)

A patient with severe neutropenia due to Felty's syndrome had limited stage small cell lung cancer, resulting in a therapeutic dilemma. Because he had a cellular bone marrow and no history of recurrent infection, we decided to administer full doses of cytotoxic chemotherapy with curative intent. A durable remission was achieved without complication, despite persistent severe peripheral neutropenia. It may be possible to administer aggressive chemotherapeutic regimens to selected patients with Felty's syndrome when there is the potential for cure of malignant disease.
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PMID:Small cell lung cancer in a patient with neutropenia due to Felty's syndrome. 255 13

This prospective study documents the haematological responses in 300 rheumatoid patients (RA) treated with sulphasalazine (SASP) for between 1 and 9 years. It also examines the effect of SASP on the total white cell and platelet counts over 2 years in relation to disease activity in 80 RA patients. Neutropenia occurred in six (2%) (three severe--neutrophil count less than 0.8 X 10(9)/l) after 3 and 12 weeks. The drug was withdrawn in six immediately and in one patient after 21 months when the neutrophil count fell to 0.7 X 10(9)/l. An additional 11 (3.7%) developed mild or transient leucopenia between 2 weeks and 24 months, and eight continued therapy. Thrombocytopenia occurred in one patient at 18 weeks associated with other reactions. Four with Felty's syndrome developed a further fall in the total WBC associated with thrombocytopenia in two. A rise in mean cell volume was common (72%), and macrocytosis (MCV greater than 98 fl) occurred in 27 (9%). Macrocytic anaemia was rare (less than 1%). All haematological problems were reversible. In 80 patients treated with SASP for 2 years there was a significant fall in the median white cell and platelet counts at 3 months associated with improvement in disease activity.
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PMID:Sulphasalazine in rheumatoid arthritis: haematological problems and changes in haematological indices associated with therapy. 256 48

The proliferation of large granulated T lymphocytes (LGL) with neutropenia and splenomegaly can be observed in patients with rheumatoid arthritis (RA), in a similar way to Felty's syndrome. We report a female with long standing AR, xerophthalmia, xerostomia, neutropenia and LGL lymphocytosis. The phenotype of the latter was CD3+, CD8+, HNK1+. She was treated with methotrexate and corticosteroids. The genetic rearrangement study did not show monoclonality. Although LGL lymphocytosis can infiltrate several organs, salivary glands infiltration has not been reported. The development of a sicca syndrome can be a feature of LGL lymphocytosis and induce diagnostic mistakes in RA.
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PMID:[Sicca syndrome caused by large granulated lymphocytes in rheumatoid arthritis]. 262 75

We characterized neutrophil autoantigens using an immunoblotting technique with antibodies obtained from patients with autoimmune neutropenia. These results were correlated with serologic characterization of the antibodies, using indirect immunofluorescence and leukoagglutination. Of the 17 sera immunoblotted, 16 showed discrete bands in the molecular weight range of 30 to 112. Three patients with Felty's syndrome reacted with an antigenic target of 80 to 84 Kd molecular mass, a finding not seen in any of the other patients studied. By serologic testing, none of the autoimmune sera showed serologic specificity for any known neutrophil-specific alloantigen. Using an anti-NA-1 serum, we identified antigenic targets at 40, 50, and 101 Kd in both NA-1-positive and NA-1-negative neutrophils. Ten of 17 autoimmune sera showed reactivity in this corresponding range. These studies demonstrate that immunoblotting may be used to identify antigenic targets in autoimmune neutropenia and may suggest a specificity of these antibodies not definable by serologic techniques. Correlation of immunoblot reactivity with disease states associated with immune neutropenia may be useful in the study of the pathogenesis of the different forms of autoimmune neutropenia.
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PMID:Immunoblotting characterization of neutrophil antigenic targets in autoimmune neutropenia. 267 17

A young female patient who had documented seropositive rheumatoid arthritis and was treated consecutively with aspirin, diclofenac, and gold salts was admitted years later for severe neutropenia. On examination she had, in addition, fever, positive rheumatoid factor, reversible swan-neck deformity of the fingers but otherwise normal joint findings. The patient responded to prednisone therapy. This case would appear to be a most unusual variant of Felty's syndrome.
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PMID:Felty's syndrome without concomitant arthritis: a variant. 275 10

We evaluated the clinical and hematologic response to methotrexate (MTX) in 4 women with Felty's syndrome (FS) who had had neutropenia for 1-3 years. Since immune complexes or antineutrophil antibodies are implicated in the pathogenesis of the neutropenia of FS, we also measured both direct and indirect levels of neutrophil-reactive IgG. All 4 patients showed a prompt and dramatic increase in neutrophil counts within 1-2 months of starting MTX therapy. In 3 patients, the symptoms of arthritis also improved; in the fourth patient, arthritis worsened. Recurring infections ceased in 3 patients. Neutrophil-reactive IgG levels, which were elevated in all patients prior to treatment, decreased toward normal while the patients were receiving MTX therapy. We conclude that MTX is effective in treating the neutropenia of FS, in part by lowering neutrophil-reactive IgG.
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PMID:Reversal of neutropenia with methotrexate treatment in patients with Felty's syndrome. Correlation of response with neutrophil-reactive IgG. 292 54

Neutrophils of 31 patients with neutropenia and rheumatoid arthritis (RA) have been studied to assess their ability to generate superoxide radicals (O-2) on activation. Seventeen patients had classical Felty's syndrome and 14 presumed chrysotherapy related neutropenia. Results were compared with those from age and sex matched controls with uncomplicated RA and from normal subjects. Neutrophils from patients with Felty's syndrome had a significantly reduced ability to generate superoxide radicals when compared with the other three groups. In addition, serum levels of IgG polymorphonuclear leucocyte binding activity (IgG PBA) were also raised in the group with Felty's syndrome. A statistically significant inverse correlation existed between O-2 generation and IgG PBA. It is concluded that neutrophil reactive IgG may have an important role in both quantitative and qualitative defects in neutrophil function in Felty's syndrome.
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PMID:Depressed superoxide radical generation by neutrophils from patients with rheumatoid arthritis and neutropenia: correlation with neutrophil reactive IgG. 302 89

Mononuclear cells expressing Fc gamma receptors that form Facb rosettes are increased in the peripheral blood of patients with rheumatoid arthritis compared with controls. Healthy individuals with a positive skin response to tuberculin showed a marked increase in numbers of circulating Facb-R+ cells three days after challenge, returning to baseline after seven days. No response was observed in subjects showing a negative skin test. A similar increase in Facb-R+ cell numbers was measured after intramuscular injection of another specific antigen, tetanus toxoid. In addition to this enhancement of Facb-R+ cell numbers, evidence has been obtained that these cells are in an activated state postimmunisation as judged by acquisition of low density and increased expression of class II MHC antigens. Apparently identical changes in Facb-R+ cell numbers and activation may be induced in vitro either by culturing sensitised mononuclear cells with specific antigen for three days or by an overnight incubation of normal cells with gamma-interferon (gamma-IFN). By analogy, therefore, the increased numbers of Facb-R+ cells in patients with rheumatoid arthritis are probably induced by gamma-interferon generated as part of an antigen driven immune response. In this context it is interesting that patients with Felty's syndrome, in whom neutropenia increases susceptibility to infections leading to the possibility of further stimulation of the immune system by micro-organisms, have particularly high levels of circulating Facb-R+ cells.
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PMID:Lymphocytes bearing Fc gamma receptors in rheumatoid arthritis. IV. Increased numbers and activation of Facb-R+ cells after immunisation of healthy individuals. 309 96

Felty's syndrome (FS) refers to the occurrence of rheumatoid arthritis, splenomegaly, and neutropenia. A subset of these patients has recently been described with a chronic T cell leukemia of large granular lymphocytes (LGCL). To examine the spectrum of lymphocyte abnormalities in FS and LGCL, we examined phenotypic and genotypic properties of lymphocytes from eight FS patients. In two of these FS patients, we observed an elevated proportion of T cells with an unusual phenotype (CD3+/Leu-7+/Leu-8-/CR3+) (46 +/- 5% of mononuclear cells). The FS lymphocytes had large granular morphology on Wright-Giemsa stain and were active in antibody-dependent cellular cytotoxic activity. This phenotype, morphology, and activity was similar to LGCL patients except that the latter T cells additionally expressed the Fc-IgG receptor recognized by monoclonal antibody Leu-11 (CD 15). In the remaining six FS patients, the proportion of CD3+/Leu-7+/CR 3+ T cells was only 10 +/- 8%, which was not significantly different from age-matched normal subjects (6.6 +/- 2.2%). To determine the clonality of T lymphocytes in FS and LGCL, we examined DNA for rearrangements of the T cell antigen receptor beta-chain (Ti beta) and gamma-chain (Ti gamma) genes by using Southern blotting techniques. We found a clonal rearrangement of the Ti beta 1 and Ti gamma genes in both LGCL patients. In contrast, no clonal rearrangements of Ti beta or Ti gamma genes were detected in lymphocytes from the FS patients. These results indicate that FS patients are heterogeneous in their phenotype and that one subset exhibits polyclonal expansion of an unusual lymphocyte subset.
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PMID:Comparison of T cell receptor gene rearrangements in patients with large granular T cell leukemia and Felty's syndrome. 310 95

Large cell granulocytic leukemia (LCGL) or proliferative lymphocyte T gamma disease, characterized cytologically by the presence of lymphocytes with intracytoplasmic azurophil granules, raises the problem of whether or not it is monoclonal in character. However, although it may resemble a chronic lymphoid T leukemia or Felty's syndrome, it differs by the constant finding of infiltration of the splenic red pulp by large granular lymphocytes. Studies of their immunologic phenotype and functional activity produce heterogeneous results. The disease course varies considerably: the serious nature of the infections, knowledge of the physiopathologic mechanism of the neutropenia and the importance of the tumoral syndrome could represent therapeutic indications the modalities of which have still to be defined.
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PMID:[Heterogeneity of large granular lymphocyte leukemia. 2 cases]. 320 33


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