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Query: UMLS:C0027947 (neutropenia)
 17,527 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neutropenia in association with a lupus-like illness that developed after the introduction of tocainide therapy is described. The mechanism of drug-associated neutropenia and the manifestations of drug-associated lupus are briefly discussed.
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PMID:Tocainide-associated neutropenia and lupus-like syndrome. 313 57

A group of 12 patients with rheumatoid arthritis (RA) with anti-Ro antibody were compared to an age and sex matched group with RA. Both had similar articular manifestations but the anti-Ro positive patients had more severe extraarticular as well as serological abnormalities. Vasculitis, liver abnormalities, neutropenia and circulating immune complexes were prominent while sicca features were not. Treatment with remission inducing drugs failed frequently without increased toxicity. Two patients with classical RA with anti-Ro developed subacute cutaneous lupus erythematosus and anti-dsDNA. Our observations suggest that the presence of anti-Ro in RA sera may be a marker for a small but significant subset of rheumatoid disease.
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PMID:Clinical significance of anti-Ro(SSA) antibody in rheumatoid arthritis. 325 83

Neutropenia is a life-threatening sequel of hematological disorders and a dominant factor limiting the dosage of cytotoxic chemotherapy. The role of the neutrophil is of such importance in defence against microbial invasion that measures that modify the behaviour of residual hemopoietic tissue to promote a modest increase in neutrophils, can confer considerable benefit by reducing the frequency and severity of infection. Such a change can be mediated in bone marrow depression by diversion of more progeny of immature precursors into the neutrophil series, or by enhancement of the stimulatory drive operating on neutrophil production. The former effect can be achieved by hypertransfusion of red cells to reduce the demand on the limited precursor population for cells of the erythroid series. The latter effect can be achieved by administration of lithium carbonate. Neutropenia caused by autoimmune injury to the neutrophil series can also be successfully modified by measures which suppress the underlying immune dyscrasia or the function of the reticulo-endothelial system. Corticosteroid administration and splenectomy can be helpful in certain specific types of neutropenia. Administration of cyclophosphamide and azathioprine has both mutagenic and marrow suppressive potential, but can induce remissions in severe chronic isolated neutropenia and in systemic lupus erythematosis.
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PMID:New concepts in management of neutropenia. 385 79

Activated complement components and immune complexes cause neutrophil (PMN) aggregation in vitro and in vivo, as in dialysis-induced neutropenia and adult respiratory distress syndrome. To investigate the possible role of PMN aggregation in systemic lupus erythematosus (SLE), we studied the capacity of 59 sera from 53 patients to induce aggregation of normal PMN in vitro. Neutrophil aggregating activity (NAA) was present in the sera of 26 of 28 patients with active SLE. The mean NAA in this group was significantly greater than that found in 13 patients with inactive SLE, 20 patients with rheumatoid arthritis, and 17 normal controls. In patients with SLE there was a positive correlation between disease severity and the quantitative measure of NAA. NAA did not correlate with serum C3 or C4 nor with the presence or absence of circulating immune complexes. High levels of NAA were particularly characteristic of central nervous system lupus. These data suggest that the formation of intravascular leukoaggregates may contribute to morbidity in SLE.
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PMID:Neutrophil aggregation induced by sera from patients with active systemic lupus erythematosus. 684 25

We applied a radiolabeled antiglobulin test to a study of patients with a variety of neutropenic disorders. After defining the nature of the interaction of radiolabeled anti-IgG with the neutrophil, we studied 16 patients with neutropenia of uncertain etiology and adequate bone marrow granulocyte precursors. Twelve of these 16 patients had increased neutrophil-associated IgG (PMN-IgG). Patients with the highest levels of PMN-IgG had the lowest neutrophil counts. The majority of patients with neutropenia and increased PMN-IgG had an underlying immunologic disorder that included immune thrombocytopenic purpura in 5 patients and autoimmune hemolytic anemia in 1 patient. In some patients, elevated PMN-IgG preceded other evidence for immunologic disease. The direct antiglobulin test helped to distinguish neutropenic patients with increased PMN-IgG both from patients with neutropenia due to a known nonimmune disorder and from nonneutropenic patients with rheumatoid arthritis or systemic lupus erythematosis. Each of four patients with increased neutrophil-associated IgG treated with systemic corticosteroids responded clinically with an associated fall in neutrophil IgG and a rise in the circulating neutrophil count. The radiolabeled antiglobulin test appears useful in defining a subpopulation of patients with neutropenia due to an underlying immunologic disorder.
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PMID:Granulocyte-associated IgG in neutropenic disorders. 697 94

A modification of the microcytotoxicity assay of Terasaki & McClelland was used to screen for serum inhibitors of myeloid progenitor cells (CFU-C). Sera from 104 patients with neutropenia or bone marrow failure and from 104 controls, including 30 normal subjects and 74 patients with various disorders or multiple transfusions, were studied. Inhibitors of CFU-C were found in 19 of the 104 patients with neutropenia or marrow failure: three with acquited neutropenia and 16 with aplastic anaemia. In 17 of the 19 patients the inhibitor was either complement dependent or chavincingly associated with clinical remission of disease in one patient. In another patient, the inhibitor was an IgG antibody directed against non-HL1 antigens present on CFU-C of all 50 normal target marrows tested; however, it was not clearly autoreactive. Inhibitors were also found in one of nine patients with lupus erythematosus, one with breast carcinoma, one patient with renal transplant rejection, and six of 46 multiply transfused patients without marrow failure. Our findings indicate that most serum inhibitors are associated with multiple transfusion: convincing evidence of pathogenetically important autoreactive antibody was present in only one of 19 patients with inhibitors.
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PMID:Serum inhibitors of myelopoiesis. 737 94

Monthly intravenous cyclophosphamide (IVCY) has been a recommended therapy for severe lupus nephritis or neurological flare-ups in lupus patients. But the optimal treatment regimen and duration remains unknown. We report our experience in an open study of 37 patients treated with monthly IVCY. Thirty-four women and 3 men, mean age 35.5 with a mean disease duration of 59 months, with a mean 5.7 ACR criteria for SLE were analysed. 27 (group I) had lupus nephritis (OMS Class III or IV) and 10 had neurological involvement (group II). In group I, after six months of IVCY, a significant improvement was noticed in the UCH-Middlesex clinical index (2.9 pts vs 7.8), the proteinuria (3.12 g/d vs 5.4), complement and split fractions (CH50 98.4 vs 48.9%; C3 877 vs 600 mg/l; C4 177 vs 128 mg/l), the level of anti-DNA antibodies (67.5 vs 775 UI/ml) and the daily dose of steroids (22 vs 44 mg/d). Kidney biopsies showed a reduction of the activity index despite a slight increase of the chronicity index (4.1 vs 6.3 pts and 5.5 vs 3.6 pts). Those results were not maintained at medium and long term. Moreover five patients presented with worsening of renal function during IVCY treatment and two patients relapsed after the end of the treatment. In group II significant improvement was noticed at six months concerning the clinical index (1.77 pts vs 7.17) and the daily dose of steroids, 3 patients died because of cerebral vasculitis refractory to IVCY. Adverse effects are frequent: infectious (25 among 20 patients), hemorrhagic cystitis (2 events in 1 patient), gastrointestinal side effects were common (12/37 patients). Were also noticed: neutropenia (5/37), transient amenorrhea (4/28), drug induced menopausis (2/28). Overall mortality is important (7/37), uneffectiveness of IVCY was noticed in 5 patients, flares occurred in 8 patient during or after stopping treatment. IVCY seems efficacious if given at the very beginning of the flare. Its usefulness is obvious at six months among clinical and biological data in patients with severe lupus nephritis or neurological flare. It seems that long term outcome on the renal function is not modified.
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PMID:[Treatment of acute systemic lupus erythematosus with intravenous infusions of cyclophosphamide. Value and limitations]. 802 84

Kikuchi's disease is rare necrotic histiocytosis of the lymph nodes. Since the first description in Japan in 1972 by Kikuchi, several cases have been reported. The disease occurs most frequently in young women. Manifestations include enlargement of the cervical lymph nodes, sometimes with fever, and often associated with other non-specific clinical signs. Blood chemistry, including immunologic tests, are often normal but neutropenia and raised erythrocyte sedimentation rate have been reported. Diagnosis relies on the histological examination of lymph node biopsies. Generally the clinical course is favourable in 3-4 months. Secondary systemic lupus erythomatosus may develop and require regular follow-up. The aetiology of this rare disease is still unclear although certain observations would favour an immunological process. Very rare in France, we report a case of Kikuchi's disease in a pregnant woman and present a review of the literature.
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PMID:[Kikuchi's disease. Apropos of a case. Review of the literature]. 809 19

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

Our study describes the presence of antineutrophil cytoplasmic antibodies (ANCA) in a group of different pathologies comprising 101 patients. Rheumatoid arthritis, systemic lupus erithematosus, idiopatic neutropenia, acute post-streptoccocal glomerulonephritis, minimal change nephrotic syndrome, Downs syndrome, adult periodontitis, tumoral calcinosis, monoartheritis and lipodystrophy were investigated for ANCA, through indirect immunofluorescence and an indirect solid-phase immunoassay (ELISA). Our results show the pattern of distribution of ANCA in the diseases investigated, and allowed us to make the first description of ANCA in diseases such as Downs syndrome, acute post-streptococcal glomerulonephritis and adult periodontitis. The high percentage of reactivity for ANCA detected in adult periodontitis, raise important questions about the possibility of reporting inaccurate percentages of positivity for some diseases, due to the presence of a concurrent disease such as adult periodontitis.
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PMID:[Antineutrophil cytoplasmic antibodies (ANCA): study of their presence in diseases not associated with arteritis]. 871 20


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