Cases reported "Agranulocytosis"

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1/10. Recurrent bacterial infections in four siblings with neutropenia, eosinophilia, hyperimmunoglobulinemia A, and defective neutrophil chemotaxis.

    Four siblings with recurrent bacterial infections, neutrophil chemotactic defect, neutropenia, and eosinophilia were studied. During periods of infection the peripheral neutrophil count increased to normal, while the eosinophilia disappeared. In addition, these children had high levels of serum IgA and poor antibody responses to tetanus and polio vaccinations. A defect in cell-mediated immunity was demonstrated by an absent or weak reactivity to various skin test antigens and by abnormal lymph node histology. Thus these siblings had an unusual combination of defective inflammatory response and immunologic abnormalities.
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keywords = bacterial infection
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2/10. Differential effects of granulocyte-macrophage colony-stimulating factor and granulocyte colony-stimulating factor in children with severe congenital neutropenia.

    Severe congenital neutropenia (SCN) is a disorder of myelopoiesis characterized by severe neutropenia secondary to a maturational arrest at the level of promyelocytes. We treated five patients with SCN with recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) for 42 days and subsequently, between 1 and 3 months later, with rhG-CSF for 142 days. The objective was to evaluate the safety and ability of these factors to elicit a neutrophil response. rhGM-CSF was administered at a dose of 3 to 30 micrograms/kg/d (30 to 60 minutes, intravenously). In all patients, a specific, dose-dependent increase in the absolute granulocyte counts was observed. However, in four patients this increase was due to an increase in eosinophils, and in only one patient it was due to an increase in the absolute neutrophil counts (ANC). Subsequently, all patients received rhG-CSF at a dose of 3 to 15 micrograms/kg/d subcutaneously. In contrast to rhGM-CSF treatment, all five patients responded to rhG-CSF during the first 6 weeks of treatment with an increase in the ANC to above 1,000/microL. The level of ANC could be maintained during maintenance treatment. In one patient, the increase in ANC was associated with an improvement of a severe pneumonitis caused by peptostreptococcus and resistant to antibiotic treatment. No severe bacterial infections occurred in any of the patients during CSF treatment. All patients tolerated rhGM-CSF and rhG-CSF treatment without severe side effects. These results demonstrate the beneficial effect of rhG-CSF in SCN patients.
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ranking = 0.2
keywords = bacterial infection
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3/10. Capnophilic and anaerobic bacteremia in neutropenic patients: an oral source.

    The currently accepted empiric antibiotic therapy for bacterial infections in neutropenic patients may not cover the possibility of capnophilic and anaerobic bacteremia. Many of these infections develop in patients with severe mucositis or periodontitis, and the type of organisms recovered also suggests an oral source of infection. We present two cases of bacteremia in neutropenic patients who had been empirically treated with ceftazidime and piperacillin plus amikacin. In the first case a beta-lactamase-producing strain of capnocytophaga ochracea was isolated; in the second case bacteremia was due to a mixture of leptotrichia buccalis and fusobacterium nucleatum. These observations emphasize the necessity for a reevaluation of the possible use of antimicrobial agents active against beta-lactamase-producing capnophilic organisms and anaerobic bacteria during empiric therapy in neutropenic patients with an oral source of infection.
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ranking = 0.2
keywords = bacterial infection
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4/10. Infantile agranulocytosis with survival into adolescence: periodontal manifestations and laboratory findings. A case report.

    A case of infantile agranulocytosis with survival into adolescence is presented. The polymorphonuclear leukocyte is considered an important source of lysosomal enzymes in gingival crevicular fluid, and evaluation of connective tissue-degrading enzymes in the fluid was performed. The activity of beta-glucuronidase, a ground substance-degrading enzyme that may serve as a marker for polymorphonuclear leukocytes, was markedly reduced in the fluid compared to samples from systemically healthy adults with periodontitis. The activities of the ground substance-degrading enzyme arylsulfatase, and collagenase, were in the low-normal range. The plaque microbiology, as characterized by dark-field microscopy and selective culturing, was consistent with advanced periodontitis. A review of the medical history revealed a series of bacterial infections since infancy. Improvement in the systemic health of the patient occurred at about the age of 15, and the intake of antibiotics to control infections was correspondingly reduced after this time. An exacerbation of the patient's periodontal disease, as evaluated by loss of alveolar bone on radiographs, occurred 1 to 2 years later. The progression of periodontal disease observed in this patient was apparently associated with the withdrawal of antibiotics administered for control of systemic (nonoral) infections.
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ranking = 0.2
keywords = bacterial infection
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5/10. Analysis of antibody to neutrophils associated with autoimmune neutropenia: possible recognition of Fc-receptor-related molecules.

    The serum of a 50-year-old male with neutropenia and a bout of bacterial infection was studied. Anti-neutrophil IgG antibody was detected by indirect immunofluorescence using a laser flow cytometry system. Purified IgG from our patient's serum did not inhibit chemotaxis of neutrophils, but inhibited rosette formation of neutrophils with ox red blood cells (ORBC) coated with anti-ORBC rabbit IgG dose-dependently. Surface iodination of neutrophils followed by their immunoprecipitation by purified IgG and sodium dodecylsulfate polyacrylamide gel electrophoresis showed a single band that corresponded to 45 kilodaltons. Possibly the IgG antibody in our patient's serum recognizes molecules related to Fc receptors.
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ranking = 0.2
keywords = bacterial infection
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6/10. Long-term remission of immune neutropenia after treatment with high-dose intravenous 7S-immunoglobulin.

    In a 61-year-old woman recurrent bacterial infections were caused by immune neutropenia which was resistant to high-dose cortisone and plasmapheresis. However, high-dose intravenous 7S-immunoglobulin therapy (30 g for 6 days) induced a long-term remission for 35 months. This treatment modality is safe and should be used as a first-line agent.
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ranking = 0.2
keywords = bacterial infection
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7/10. Pseudoleukemia due to infection. A case report.

    A 29-year-old white female developed fever, vomiting, diarrhea, and hypovolemic shock. Twenty-four hours after resection of intraperitoneal adhesions she had granulocytopenia and leukopenia with a marked "left shift"; a bone marrow aspirate was interpreted as showing acute non-lymphocytic leukemia. The clinical presentation made this diagnosis unlikely and the subsequent course indicated that this was a reaction to bacterial infection.
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ranking = 0.2
keywords = bacterial infection
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8/10. Manifestations and treatment of periodontal disease in a patient suffering from cyclic neutropenia.

    A case of severe periodontitis in a young man suffering from cyclic neutropenia is reported. The periodontal status as evaluated from oral radiographs at the age of 13 reveals advanced periodontal breakdown around the molars and the incisors in both jaws. At the age of 21, when clinical examination was performed, all teeth were found to be periodontally involved. The treatment was divided into three phases: (1) a causative treatment phase (oral hygiene phase) aimed at achieving the highest possible level of oral cleanliness, (2) a corrective phase including periodontal surgery and prosthetic rehabilitation and (3) a maintenance phase aimed at prevention of recurrence of periodontal disease. The oral hygiene phase was markedly extended both in time (2 years) and intensity. Several reexaminations were carried out during the course of this phase to evaluate the patient's ability to exercise proper plaque control and the response of the tissues to treatment. The progression of the periodontal disease could be arrested and the prosthetic treatment indicated was successfully performed. Following the corrective treatment phase a maintenance care program was designed including frequent recall appointments in order to prevent disease recurrence. Four years after active treatment no further periodontal breakdown could be observed as evaluated from pocket depth, attachment level and bone height measurements. The result of the treatment of this patient shows that treatment, designed to eliminate plaque infection, is effective also in an individual with defective host defense against bacterial infections, but the demand on the patient's cooperation must be extraordinary high as well as the demand on the effectiveness of the maintenance care program.
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ranking = 0.2
keywords = bacterial infection
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9/10. Suppression of granulopoiesis by t-lymphocytes in two patients with disseminated mycobacterial infection.

    Two patients with disseminated mycobacterial infection presented with severe neutropenia and hematopoietic failure. Marrow cells were obtained from each patient and were cultured in methylcellulose before and after the removal of mononuclear phagocytes, t-lymphocytes, or both from the marrow cell suspension. Glucocorticosteroid-resistant t-lymphocytes markedly inhibited granulopoiesis, but mononuclear phagocytes did not. indomethacin inhibition of prostaglandin synthesis did not influence suppression of colony growth by the inhibitory lymphocytes. In the one patient who responded favorably to antituberculous therapy, the in-vitro T-lymphocyte inhibition of granulopoiesis disappeared as the neutropenia resolved. Thus, in some patients with disseminated mycobacterial infection, clinical bone marrow failure may be mediated, at least partly, by t-lymphocytes that suppress hematopoiesis.
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ranking = 1
keywords = bacterial infection
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10/10. Acquired cyclic neutropenia: successful treatment with prednisone.

    A previously healthy woman developed severe, periodic neutropenia after ingestion of phenylbutazone. Oscillations in the monocyte count and hemoglobin concentrations also occurred. The neutropenic episodes were associated with severe bacterial infections requiring hospitalization. lithium induced a transient interruption in the neutropenia, but continued use was ineffective. prednisone in a dosage of 100 mg daily successfully interrupted the neutrophil cycling and prevented infection. The patient has remained in remission on 10 mg of prednisone on alternate days.
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ranking = 0.2
keywords = bacterial infection
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