Cases reported "Neutropenia"

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1/13. Congenital neutropenia. Report of a case and a biorationale for dental management.

    Congenital neutropenia is characterized by a marked decrease in or lack of circulating PMN's in children with no prior history of drug intake. The neutropenia is persistent and the clinical course is one of early onset of severe, recurrent, and eventually fatal infections. bone marrow studies show a maturation arrest of neutrophilic precursors. Because of their greatly increased susceptibility to infection, patients with congenital neutropenia present a difficult dental management problem. A case of congenital neutropenia has been presented, as well as a biorationale for dental treatment. On the basis of reports in the literature, the following recommendations for the management of patients with congenital neutropenia are made: 1. The prevention and control of infection and the interception of dental disease before surgical intervention becomes necessary should be the overriding considerations in the management of patients with congenital neutropenia. 2. The carious breakdown of teeth should be prevented by the daily application of a 0.4 per cent stannous fluoride gel in addition to oral hygiene and limitation of sucrose intake. 3. Periodontal therapy should be palliative only, since alveolar bone loss is progressive despite frequent oral hygiene instruction and prophylaxis. The goal of periodontal therapy for patients with congenital neutropenia should therefore be a decrease in gingival inflammation to make the patient's mouth more comfortable and to slow down alveolar bone loss. Periodontal surgery is contraindicated. 4. bacteremia and subsequent septicemia should be prevented since a minor infection can become life threatening in patients with congenital neutropenia. The patient should rinse for 30 seconds and the gingival sulci should be irrigated with a phenolated antiseptic mouthwash prior to all dental manipulations of the soft tissue. This will significantly reduce the incidence of bacteremia. 5. Surgery should be avoided if at all possible because of the high risk of post-operative infection. All surgery sholld be performed in the hospital, and the patient should be given antibiotics as determined by his physician. Primary closure should be done with fine polyglycolic acid sutures to reduce the chance of infection. If postoperative infection can be prevented, wound healing will progress normally despite the complete absence of PMN's.
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2/13. Colorectal cancer: dilemmas regarding patient selection and toxicity prediction.

    Irinotecan (Campto, Rhone-Poulenc Rorer) is probably the most studied drug used as second-line treatment for colorectal cancer. Its main disadvantages are toxicity and cost. Delayed diarrhea and neutropenia are the most common toxic side effects, both of which can usually be predicted, by knowing the criteria for patients who are at increased risk for those side effects. These criteria include poor performance status (>2), bulky disease, previous abdominal-pelvic irradiation, hyperleukocytosis and increased bilirubin >1.5 x normal upper range. There are some other less common toxic effects of irinotecan, such as pneumonitis, cardiac arrhythmia, paralytic ileus, liver dysfunction, tumor lysis syndrome. While these side effects are very rare, physicians should be able to recognize them, because the number of patients being treated with irinotecan is increasing. The authors report four cases of probable irinotecan-related toxicity with fatal outcome in all 4 patients. Two of these 4 patients were not in the known risk categories for irinotecan toxicity. One patient died with signs of hepato-renal syndrome, the other with signs of rapid tumor lysis-like syndrome. Two other patients with bulky disease and performance status 2, had increased urea, creatinine and bilirubin serum levels after irinotecan administration, that could not be explained as manifestation of disease progression only. Data on all uncommon irinotecan toxic effects should be gathered and analyzed so that toxic effects, other than diarrhea and neutropenia, are better defined and predicted.
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3/13. Toxic effects associated with consumption of zinc.

    A 27-year-old man with a history of acne presented to his primary care physician because of fatigue and dyspnea on exertion of 4 weeks' duration. He was remarkably pale, orthostatic pulse changes were noted, and a systolic ejection murmur was heard. The patient had profound anemia (hemoglobin concentration, 5.0 g/dL) and neutropenia (neutrophil count, 0.06 x 10(9)/L); he was admitted for further evaluation. A detailed inquiry into his medication history revealed that he was taking several vitamins and zinc gluconate, 850 to 1000 mg/d for 1 year (US recommended daily allowance, 15 mg), as therapy for acne. A zinc toxic and copper-deficient state was confirmed by laboratory studies. The patient was treated with intravenous copper sulfate, followed by 3 months of oral therapy. The complete blood cell count, serum copper level, and serum zinc level returned to normal.
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4/13. A neutropenia suggesting an interaction between valacyclovir and mycophenolate mofetil.

    Mycophenolate mofetil (MMF) is a drug which decreases the frequency of renal transplantation rejection. However, cytomegalovirus infections are a common feature of this treatment leading the physicians to prescribe antiviral prophylactic drugs like valacyclovir. During this association, neutropenia occur and the cause of this adverse effect is difficult to define. This report presents a case of neutropenia in a woman treated with MMF and valacyclovir. As the duration of the valacyclovir treatment exactly corresponds to the neutropenia duration, and the mycophenolate trough levels increased with the neutrophil count, the responsibility of this neutropenia was ascribed to valacyclovir. However, an examination of the literature for cases of neutropenia led to the suspicion of an interaction between MMF and valacyclovir. Mycophenolate may increase intracellular concentrations of valacyclovir up to haematotoxic levels. This mechanism may explain the interaction and further research is needed to confirm this interaction.
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5/13. noma-like gangrenous cheilitis in a child with cyclic neutropenia associated with myeloperoxidase deficiency.

    An unusual case of cyclic neutropenia and hereditary myeloperoxidase deficiency complicated by noma-like gangrenous cheilitis is described. klebsiella pneumoniae and candida albicans were cultured from the involved area. We present this case to increase physician awareness of the possible association of both quantitative and qualitative defects of neutrophils and to stress the importance of the early and effective management of skin infections that can rapidly progress to severe sequelae when associated with profound neutropenia.
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6/13. Randomization, informed consent and physicians' communication skills in pediatric oncology: a delicate balance.

    parents asked to consent to a child's randomization in a pediatric cancer clinical trial are often unprepared to grasp the implications of this scientifically crucial but seemingly unfair process. physicians must adopt specific communication skills to engage families in open dialogue from the outset in order to elicit truly shared informed consent. Starting from the case of a family with an only child affected by disseminated neuroblastoma, we wish to comment on the problems surfacing in the informed consent process for treatment and research in pediatric oncology that implicate an understanding of bioethical issues and psychological principles. Although the outcome of childhood cancer has improved dramatically over the last 30 years, with overall survival rates now exceeding 70%, there are regretfully still types and stages of cancer carrying a very high risk of death that urgently require new clinical strategies. The response to this need has been the design of experimental protocols that often entail randomized controlled trials (RCT). A large number of these trials concern stage IV neuroblastoma, acute leukemia, rhabdomyosarcoma, and other types of childhood cancers presenting great heterogeneity both in terms of localization and responsiveness to therapy. Most trials for disease relapses also include one or more randomizations. The scientific motivation justifying an RCT is the need to compare and evaluate an innovative protocol (or part thereof) with reference treatment modalities. Nevertheless, the process brings to bear the ethical dilemma of having to weigh the needs of the single afflicted child against the benefit which may ensue for a much larger patient community.
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7/13. carbamazepine. What physicians should know about its hematologic effects.

    Transient leukopenia and, less commonly, neutropenia may occur with carbamazepine therapy. Discontinuation of therapy is usually not indicated unless symptoms are severe, persistent, or accompanied by infection. patients with a low leukocyte or neutrophil count before treatment may be at increased risk for carbamazepine-induced leukopenia or neutropenia. Careful monitoring of blood counts, particularly during the first month of therapy, is essential. The frequency of monitoring can be determined on an individual basis. If a hematologic abnormality develops, the frequency of monitoring should be increased, especially if carbamazepine is not discontinued. Only when the neutrophil count falls below 500/mm3 does a severe risk of infection exist.
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8/13. Brief communication: Legionnaire's disease successfully treated in acute myelocytic leukemia during severe neutropenia.

    A patient with acute nonlymphocytic leukemia developed progressive lung infiltrates and unremitting fevers during a profound neutropenic state. Legionnaire's disease was diagnosed by simple immunologic studies and successfully treated with erythromycin. This index case alerts physicians toward a treatable infection which would not normally be susceptible to the empiric antibiotic regimens given neutropenic patients with fevers.
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9/13. Neutropenic typhlitis in adults.

    typhlitis is a necrotizing process of the cecum, occurring in neutropenic patients who are usually receiving combined chemotherapy. The atonic cecum is affected because of its poor arterial perfusion, the presence of colonic bacteria, and the milieu of immunosuppression. It is a serious but not inevitably fatal complication of the patient's basic illness. Resection of necrotic bowel is necessary. Because this will usually be done on an emergency basis in a very ill patient with unprepared bowel, a two-stage procedure rather than primary anastomosis is advisable. Surgical results may be better in adult than pediatric patients. The incidence of the problem has probably increased since the advent of aggressive drug therapy of the leukopathic states. Judging from the low number of reported adult cases and the apparent inaccuracy of preoperative diagnosis, there may be inadequate physician awareness of typhlitis.
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10/13. neutropenia induced by semisynthetic penicillin.

    The semisynthetic penicillins, like the parent drug penicillin g, are remarkably safe drugs. However, a significant number of patients have been reported in whom profound neutropenia has developed. This is a side effect of these drugs not appreciated by a significant number of physicians. I report two more cases of neutropenia due to nafcillin and review the literature on neutropenia induced by semisynthetic penicillin. The relative risk of each individual drug is evaluated, and the two proposed mechanisms of production of the neutropenia are discussed.
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