Cases reported "Neutropenia"

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1/10. Benign chronic neutropenia with abnormalities involving 16q22, affecting mother and daughter.

    We report a case of familial, chronic, benign neutropenia in a 17-year-old female showing (1) the spontaneous expression of a heritable rare fragile site at 16q22 and (2) a deletion at the same region. The del(16)(q22), which most likely originated from the fragile site, was the main clonal abnormality detected in the patient's bone marrow cells, whereas a few cells with either del(16)(q22) or fra(16)(q22) were seen in the patient's peripheral blood. Interestingly, the del(16q) was also detected in the patient's uncultured cells, as demonstrated by FISH, excluding an in vitro origin of the del(16q) during culture. The bone marrow was hypocellular with decreased neutrophils and their precursors. Absolute neutrophil counts ranged from (0.62 to 1.24) x 10(9)/L with a median value of 1.02 x 10(9)/L. The patient had a more severe neutropenia than her mother, which correlated with the presence of more cells with del(16q) in the marrow. The patient's mother, who was also diagnosed with neutropenia, revealed only a few cells with the rare fra(16)(q22) in her peripheral blood cells, whereas her bone marrow showed cells with both fra(16)(q22) and del(16)(q22), although the del(16q) was present in only 2/20 cells. Some possible candidate genes contributing to the pathogenesis of the neutropenia are discussed. Chromosome abnormalities involving the 16q22 breakpoint have been observed in myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML). In this patient, the del(16)(q22) risk factor is unknown for subsequent development of MDS or AML. Another point to consider is the need to determine the origin of a chromosome abnormality, particularly when the clinical picture does not fit the chromosome findings. Although, the observation of a constitutional structural abnormality in a mosaic form is an extremely rare event, it is somewhat different in the case of a fragile site expression, which can, as in this case, be present in some cells and not in others.
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ranking = 1
keywords = candida
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2/10. Bilateral chronic fungal dacryocystitis caused by Candida dubliniensis in a neutropenic patient.

    In recent years, candida species other than candida albicans have emerged as causes of human candidiasis, particularly in hiv-infected and other immunocompromised people. C. dubliniensis, a recently described species closely related to C. albicans, first isolated from patients with AIDS in Dublin, has been implicated as an agent of oral candidiasis in hiv-positive people. However, it has also been recovered from hiv-negative people, with clinical signs of oral candidiasis and from the genital tract of some women with vaginitis. The first case of bilateral chronic fungal dacryocystitis caused by C. dubliniensis is described in an hiv-negative woman.
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keywords = candida
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3/10. Phycomycosis infection in acute leukaemia.

    Fungal infections are increasingly being reported in patients with acute leukaemia on intensive induction chemotherapy protocols. The common fungi seen are candida, aspergillus and mucormycosis. We have seen 3 cases of mucormycosis over the last 4 years. All 3 patients had acute leukaemia-two had acute lymphoblastic and one acute myeloid leukaemia. All patients were in neutropenic phase after induction chemotherapy. Features suggestive of fungal infection were fever and development or progression of pulmonary infiltrates despite antibiotic therapy. Repeated body fluid cultures were negative in two patients. In the first patient, the diagnosis was confirmed after biopsy of a palatal mass; he was treated successfully with amphotericin-B. In two patients the diagnosis was confirmed at autopsy. A high degree of suspicion in febrile, neutropenic cancer patients on chemotherapy and early administration of amphotericin-B may improve the outcome. With dissemination, the prognosis is poor.
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ranking = 1
keywords = candida
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4/10. Hepatosplenic candidiasis: an overlooked cause of prolonged fever during recovery from an episode of neutropenia.

    Two cases of hepatosplenic candidiasis (HSC) are reported occurring after protracted episodes of neutropenia, induced by chemotherapy for acute leukemia in one case and drug hypersensitivity in the other. The disease presented with persistent or recurrent fever after correction of the neutropenia and with splenomegaly. The alkaline phosphatases were elevated. The diagnosis was strongly suggested by abdominal ultrasonography, CT scan and MRI, which showed multiple hepatosplenic defects. It was confirmed by serologic tests for candidiasis, the presence, in 1 case, of circulating candida antigens, and the rapid response to amphotericin b. The diagnosis of HSC should be considered in patients with persistent fever after an episode of neutropenia. Ideally, histologic confirmation is desirable, but this is often obtainable only by open liver biopsy, an aggressive procedure in such patients. Failing this, our 2 cases stress the diagnostic value of noninvasive imaging techniques, serological testing (in particular the discovery of circulating candida antigens) and the response to amphotericin b.
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ranking = 2
keywords = candida
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5/10. Myelokathexis associated with multiple congenital malformations: immunological study on phagocytic cells and lymphocytes.

    A 5-year-old boy was first seen at the age of 11 months when he presented with growth retardation, skeletal dysmorphisms and neutropenia. Since then he has remained leukopenic except when he has pulmonary infections with a marked leukocytosis. Despite his neutropenia, marked myeloid hyperplasia was evident on marrow smear examination; many cells showed abnormally hypersegmented nuclei, with unusual shape or pyknotic nuclei. Phagocytic cells showed impaired phagocytosis, candidacidal activity, metabolic burst and chemotaxis. Moreover, the patient's serum generated less chemotactic activity than normal serum. These data indicate a selectively complex defect of the neutrophil during differentiation associated with the presence of an inhibitor of chemotactic factors in the patient's serum.
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ranking = 1
keywords = candida
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6/10. Invasive candidal balanitis due to a condom catheter in a neutropenic patient.

    A 58-year-old man with acute leukemia had balanitis and penile ulceration due to candida tropicalis after a condom catheter was used because of urinary incontinence. This case illustrates that condom catheters can be associated with serious complications in neutropenic patients and should be used only when absolutely necessary.
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ranking = 4
keywords = candida
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7/10. Focal hepatic candidiasis: a distinct clinical variant of candidiasis in immunocompromised patients.

    Focal hepatic candidiasis is a distinct clinical variant of candidiasis in immunocompromised hosts. Although affected patients may exhibit previous evidence of extra-hepatic candidal infection, manifestations of disease at the time hepatic involvement is documented are localized to the liver. Five cases of focal hepatic candidiasis in immunocompromised patients were diagnosed antemortem in the past 15 months at our institution. Clinical, laboratory, and radiologic findings from those five patients, as well as from 20 additional cases reported in the medical literature, are reviewed. patients with focal hepatic candidiasis had fever unresponsive to antimicrobial therapy, prominent gastrointestinal symptoms and signs, elevated levels of alkaline phosphatase, and hepatic, with or without accompanying splenic, defects noted on abdominal computed tomography. liver biopsy usually revealed yeast and/or hyphal forms, but cultures were frequently negative. Response to therapy occurred in only 13 (59%) of 22 assessable patients. The apparent increasing incidence of focal hepatic candidiasis may be due to the recent use of more intensely cytotoxic chemotherapies for cancer.
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ranking = 1
keywords = candida
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8/10. Periodontal disease in three siblings with familial neutropenia.

    The periodontal status and treatment of three teenagers in a Finnish family with familial neutropenia is described. The mother was also diagnosed with neutropenia. At initial examination, the 15-year-old male and the 10-year-old female had severe periodontitis, whereas the 13-year-old male had oral ulcerations but no significant periodontal disease. The two siblings with periodontitis were treated and followed approximately 5 years. It was concluded that periodontal therapy including scaling, surgery, and use of antimicrobial agents can be successful in patients with familial neutropenia, and that such patients are not necessarily candidates for full mouth extraction. The role of granulocyte colony-stimulating factor in which was used in the treatment of these patients remains to be established.
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ranking = 1
keywords = candida
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9/10. Hepatic candidiasis: persistent pyrexia in a patient with acute myeloid leukaemia after recovery from consolidation therapy-induced neutropenia.

    fever, a frequent manifestation in acute leukaemia patients who develop treatment-induced neutropenia, usually resolves when the neutrophil count returns to normal irrespective of whether an infective agent is isolated or not. A persistent pyrexia following neutrophil recovery and associated with multiple negative microbiological cultures should signal a careful search for a deep-seated fungal infection in any leukaemic patient who is complete remission. We report here a 39-year-old Chinese man with acute myeloid leukaemia in first complete remission whose unresolved fever after recovery from consolidation therapy-induced neutropenia was ultimately confirmed to be caused by focal hepatic candidal microabscesses by an open liver biopsy.
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ranking = 1
keywords = candida
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10/10. granulocyte colony-stimulating factor-primed leukocyte transfusions in candida tropicalis fungemia in neutropenic patients.

    Optimal management of fungemia in neutropenic patients is still controversial. Several reports have already stressed the poor prognosis in invasive candidiasis (80% mortality in several reports). Therefore granulocyte transfusions would appear to be useful in the management of these infections. We report the use of rhG-CSF-primed granulocyte transfusions plus amphotericin b in two neutropenic patients who developed life-threatening systemic fungal infections. This approach was successful and both patients fully recovered from the infection.
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ranking = 4
keywords = candida
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