Cases reported "Neutropenia"

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1/119. Dizygotic twin sisters with myelokathexis: mechanism of its neutropenia.

    Dizygotic twin sisters were first found to have neutropenia at 1 year of age when evaluated for recurrent pulmonary infections. Since then they have remained neutropenic (0.05 approximately 0.5 x 10(9)/l). Despite of their neutropenia, myeloid hyperplasia was evident on a marrow smear examination, and a number of cells were hypersegmented with fine interlobular bridging with chromatin strands and cytoplasmic vacuolation. Electron microscopy showed apoptotic cells with condensed nuclei and apoptotic bodies in the cytoplasm. Although life span, hydrogen peroxide production, phagocytosis, spreading, and chemotaxis of peripheral neutrophils were normal, the survival of bone marrow neutrophils in both infants was markedly decreased when compared with that of normal bone marrow neutrophils. During the bone marrow culture apoptotic neutrophils were observed at an earlier stage in both patients than in normal controls, biochemically and morphologically. Morphology of bone marrow neutrophils in both patients resembled that of cultured control bone marrow neutrophils. Peripheral neutropenia and appearance of characteristic neutrophils in the bone marrow in myelokathexis are considered to be an expression of apoptosis of bone marrow neutrophils.
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2/119. Chronic neutropenia: Response to plasma with high colony-stimulating activity.

    A child with repeated infections was immunologically normal but was found to have neutropenia with periodic elevations of the absolute mature polymorphonuclear count at 21-day intervals. Immediately following the PMN rise, bone marrow morphology and in vitro cultures demonstrated a maturation arrest at the myelocyte stage with an increase in proliferative capacity. His cycle was not altered by infusions of normal plasma or by injections of epinephrine or typhoid vaccine. Infusion of 10 ml/kg of "stimulated" plasma from donors reactive to TV, obtained 60 minutes following immunization, resulted in an out-of-phase rise in PMN cells and clinical improvement. in vitro assays, using normal or patient marrow, detected high levels of colony-stimulating activity only in those plasma samples that were effective in the patient. These observations support a role of CSA as a physiologic regulator of granulopoiesis in man.
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3/119. Alteration of colony-stimulating factor output, endotoxemia, and granulopoiesis in cyclic neutropenia.

    Cellular and humoral factors involved in the regulation of granulopoiesis were evaluated in two patients with cyclic neutropenia by utilizing the agar-gel marrow culture technique to serially study marrow granulocytic colony-forming capacity (CFC) and the urinary output of colony-stimulating factor (CSF). CSF output varied inversely with peripheral neutrophil counts and directly with monocyte counts and evidence for infection (endotoxemia and/or staphylococcal abscesses). Following autologous infusion of one patient's plasma obtained during a period of neutropenia, increased urinary excretion of CSF occurred concomitant with increments in both marrow CFC and the proportion of granulocytic progenitor cells in dna synthesis. Neutrophil periodicity was not altered by the administration of the neutropenic plasma. These findings are consistent with the hypothesis that cyclic neutropenia is caused by a quantitatively decreased entry of stem cells or granulocytic progenitor cells into granulopoiesis.
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4/119. Primary aspergillosis of the larynx in a patient with Felty's syndrome.

    Herein we report the first case of primary aspergillosis of the larynx in a patient with Felty's syndrome. A 53-year-old man, a florist by profession, with a 12-year history of rheumatoid arthritis and on treatment with steroids, was admitted because of hoarseness, and intermittent fever of 2 weeks' duration. On admission, physical examination and laboratory data showed, among other findings, splenomegalia and neutropenia. At bone marrow examination, normal cellularity with mild dyserythropoiesis was observed. A fiberoptic laryngoscopy showed white plaques on both the true vocal cords. Both culture and microscopic examination of these lesions provided the diagnosis of invasive process by aspergillus flavus. A computed tomography of the middle ears, paranasal sinuses, and chest was normal. Thus, primary aspergillosis of the larynx and Felty's syndrome was diagnosed, and the patient was successfully treated with granulocyte colony-stimulating factor and systemic antifungal agents. Felty's syndrome, corticosteroid use, and occupational risk probably rendered our patient susceptible to Aspergillus infection.
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5/119. Identification of mycobacterium neoaurum isolated from a neutropenic patient with catheter-related bacteremia by 16S rRNA sequencing.

    A rapidly growing pigmented mycobacterial strain with an ambiguous biochemical profile was isolated from the blood culture taken through the Hickman catheter of a 9-year-old girl with acute lymphoblastic leukemia. Whole-cell fatty acid analysis showed that the best match profile was that of mycobacterium aurum, but the similarity index was only 0.217, meaning that there were no good matches between the isolate and the organisms in the database of the Microbial Identification System. The 16S rRNA gene of the mycobacterial strain was amplified, agarose gel purified, and sequenced. There were 44 base differences between the gene sequence of the isolate and that of M. aurum but only one base difference between the sequence of the isolate and that of mycobacterium neoaurum, showing that the isolate was indeed a strain of M. neoaurum by using this "gold standard." This represents the first case of M. neoaurum infection documented by 16S rRNA sequencing.
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6/119. saccharomyces cerevisiae fungemia in a neutropenic patient treated with Saccharomyces boulardii.

    A case of Saccharomyces fungemia in an 8-month-old baby affected by acute myeloid leukemia while receiving intensive chemotherapy is reported. The patient was receiving prophylaxis treatment with Saccharomyces boulardii capsules (Codex) to prevent diarrhea, which is commonly associated with this type of chemotherapy. fever spiked just the day after ending the chemotherapy course, and a strain of saccharomyces cerevisiae was isolated from blood culture although the patient was also receiving antifungal prophylaxis with fluconazole. The patient recovered, though still neutropenic, with amphotericin-B and removal of the central venous catheter. The common biochemical characteristics make it difficult to differentiate between the strain of saccharomyces cerevisiae and that of Saccharomyces boulardii with routine methods. In other cases, authors demonstrated an identity between the two strains with a more detailed analysis. These reports raise concern about the potential side effects of such biotherapeutic agents.
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7/119. typhlitis (neutropenic enterocolitis) after a single dose of vinorelbine.

    We report a case of a 50-year-old man with pretreated adenocarcinoma of the lung, who developed fatal neutropenic enterocolitis (typhlitis) after a standard dose of the cytotoxic drug vinorelbine. blood cultures were negative for all microorganisms tested for. Stool cultures were negative for enteric rods but direct examination of fresh stool revealed the presence of giardia lamblia. abdominal pain and diarrhoea developed very rapidly while the patient was only moderately neutropenic. metronidazole was prescribed without clinical benefit: the abdominal pain remained stable. The duration of neutropenia was very short (4 days). The abdominal catastrophe ending in shock occurred after complete recovery of the neutrophil count. Neutropenic colitis has been reported with increasing frequency in solid tumours after the introduction of taxanes. This complication has been observed mainly in phase I studies, near the maximally tolerated doses (MTD). The combined use of vinorelbine has recently been reported to exacerbate the toxic effects of taxane on the colon. The case presented here demonstrates that typhlitis can occur even with vinorelbine alone, used at a standard recommended dose (30 mg/m2).
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8/119. Fatal disseminated fusarium infection in acute lymphoblastic leukaemia in complete remission.

    fusarium species are increasingly recognised as serious pathogens in the immunocompromised. The outcome in the context of persistent severe neutropenia has been almost universally fatal. However, there have been several case reports of successful treatment if neutrophil recovery can be achieved. This report presents the case of a fatality that occurred despite neutrophil recovery. A 67 year old man developed disseminated fusariosis during the neutropenic phase of induction chemotherapy for acute lymphoblastic leukaemia. fusarium dimerum was isolated from blood cultures. This species is highly unusual and very few case reports exist in the literature. An initial response to amphotericin treatment coincided with neutrophil recovery but a subsequent relapse occurred, despite adequate neutrophil counts, which proved fatal. It is postulated that reseeding of the blood from an occult site, namely the right vitreum in this case, led to this secondary relapse despite achieving complete leukaemic remission.
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9/119. Usefulness of bronchoalveolar lavage for the diagnosis and treatment of refractory pneumonia in a patient with Kostmann syndrome, a severe congenital neutropenia.

    An 11-year-old girl with Kostmann syndrome developed refractory pneumonia. culture of oral discharge, throat-swab specimens, and blood could not identity the causative organism, and systemic antimicrobial therapy failed to achieve improvement. We then performed diagnostic bronchoalveolar lavage (BAL) and culture of BAL fluid (BALF) yielded pseudomonas aeruginosa. Therapeutic BAL using gentamicin produced a striking improvement of her pneumonia. Conclusion: In immunocompromised children with pneumonia, BAL helps to identify the causative organism. If the patient is unresponsive to systemic antimicrobial therapy, BAL using antimicrobial agents is also worth trying.
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10/119. scedosporium endophthalmitis: two fatal disseminated cases of scedosporium infection presenting with endophthalmitis.

    The incidence of disseminated infection with scedosporium species is increasing in patients with haematological malignancy. Two fatal cases are reported of patients with acute myeloid leukaemia and neutropenia who presented with scedosporium endophthalmitis. diagnosis of fungal infection was delayed as blood and vitreous cultures were positive only after 3 days in patient 1 and blood culture was positive at 7 days in patient 2. Despite antifungal therapy with amphotericin b and additional fluconazole in patient 2, both patients died of overwhelming fungal septicaemia. Post-mortem examination of the right globe in patient 1 showed haemorrhagic necrotizing chorioretinitis with numerous fungal hyphae in choroidal vessels, choroid, retina and vitreous. scedosporium species are often resistant to conventional antifungal therapy including amphotericin b. diagnosis is difficult and mortality in disseminated infection is high.
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