Cases reported "Lung Diseases"

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1/198. lymphomatoid granulomatosis following autologous stem cell transplantation.

    lymphomatoid granulomatosis (LYG) is a rare angio-destructive lymphoproliferative disorder (LPD) of uncertain etiology, with prominent pulmonary involvement. Recent studies indicate that LYG is an Epstein-Barr virus (EBV)-associated B cell LPD with large numbers of background reactive T lymphocytes (T cell-rich B cell lymphoma). Although the disease frequently, but not exclusively, occurs in various immunodeficiency states, it has not been reported in association with the transient immunosuppression following autologous bone marrow/peripheral stem cell transplantation (ABM/PSCT). We describe a patient who developed lymphomatoid granulomatosis of the lung approximately 2 weeks after high-dose chemotherapy and autologous peripheral stem cell transplantation for multiple myeloma. Although molecular studies showed no evidence of EBV genome in the biopsy material, the serologic profile with high IgM titers was suggestive of primary EBV infection. Complete radiologic remission occurred following reconstitution of the patient's immune response after a 2-week course of ganciclovir treatment. Despite the apparently low frequency of LPD (both LYG and EBV-associated post-transplant lymphoma) in the ABMT setting, we believe that it should be considered in the differential diagnosis of patients whose clinical course following ABMT is complicated by fevers, in the absence of an identifiable infectious process.
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keywords = bone
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2/198. Pulmonary infiltrates after cytokine therapy for stem cell transplantation. Massive deposition of eosinophil major basic protein detected by immunohistochemistry.

    interleukin-2 (IL-2), a product of activated T-cells, is now being used in a number of protocols for cancer immunotherapy. In one stem cell transplantation protocol for breast cancer, IL-2 is used together with interferon-gamma (IFN-gamma) and cyclosporine to stimulate a graft-versus-tumor response and improve the likelihood of a prolonged remission. We present the case of a patient who developed peripheral eosinophilia, perihilar infiltrates, and hypoxemia after autologous stem cell transplantation and the use of recombinant IL-2 and IFN-gamma. Histologic analysis of transbronchial lung biopsies demonstrated a few eosinophils within the bronchial submucosa. Immunostaining using antibodies directed against eosinophil major basic protein (MBP), however, revealed massive extracellular deposition of this toxic granule protein throughout the lung parenchyma. IL-2 therapy is well known to induce a peripheral eosinophilia and to be associated with the capillary leak syndrome characterized by weight gain, edema, and oliguria. The findings noted in this case report suggest that the eosinophil activation that accompanies immunologic therapy with IL-2 can result in direct toxicity to the lung and a localized vascular leak syndrome. This syndrome should be considered in the differential diagnosis of pulmonary infiltrates that occur acutely after bone marrow transplantation with cytokine augmentation.
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keywords = bone
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3/198. High-dose corticosteroid therapy for diffuse alveolar hemorrhage in allogeneic bone marrow stem cell transplant recipients.

    In a series of 74 patients with hematological malignancies undergoing allogeneic bone marrow or peri- pheral blood stem cell transplants from an HLA-identical sibling donor, four developed diffuse alveolar hemorrhage (DAH) between days 0 and 23 post transplant. diagnosis was made by the radiographic finding of diffuse bilateral lung opacities, and bloody lavage fluid on bronchoscopy. Two patients required mechanical ventilatory support. They were treated with methylprednisolone 0.25-1.5 g/day for at least 4 days with slow tapering thereafter. All patients showed an immediate response and two became long-term survivors with normal respiratory function. Two had a relapse of DAH, developed acute respiratory distress syndrome (ARDS) and died with multi-organ failure. risk factors for DAH were one or more courses of intensive chemotherapy pretransplant vs no treatment or low-dose chemotherapy (4/4 DAH vs 23/70 no DAH; P = 0.015), and second transplants (2/2 DAH vs 1/70 with no DAH; P = 0.006). These results indicate that DAH is life-threatening but is potentially reversible by prompt treatment with high doses of steroids.
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keywords = bone
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4/198. Immunohistochemical study of a patient with diffuse pulmonary corpora amylacea detected by open lung biopsy.

    The chest radiographs of an asymptomatic 58-year-old Japanese man with pulmonary corpora amylacea (PCA) revealed bilateral patchy and nodular infiltrates. lung specimens obtained by open lung biopsy were histochemically and immunohistochemically analyzed. In all sections of dissected lung tissue, hematoxylin and eosin staining revealed homogeneous eosinophilic acellular round bodies (50-100 microm in diameter) containing granular black pigments in the alveolar spaces. Some round bodies were surrounded and phagocytized by alveolar macrophages. The laminated round bodies stained positively with PAS and congo red. In addition, many of the rounded bodies contained particles which stained positively with berlin blue. Immunohistochemical staining for epithelial membrane antigen (EMA) as well as PE-10 was distinctively positive. This is a very rare case of diffuse PCA found by open lung biopsy. Immunohistochemical examination suggested that PCA consisted of pulmonary surfactant protein and epithelial membrane antigen.
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ranking = 813.97283358363
keywords = macrophage
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5/198. Diffuse alveolar hemorrhage syndrome due to 'silent' mitral valve regurgitation.

    A variety of clinical diseases are associated with diffuse alveolar hemorrhage. Although mitral valve disease can cause hemoptysis, it rarely is associated with diffuse alveolar hemorrhage at presentation. A 49-year-old woman was admitted to the hospital with the abrupt onset of fever, anemia, dyspnea, azotemia, and diffuse alveolar infiltrates. Two-dimensional echocardiography done several months earlier to evaluate atypical chest pain had been unremarkable. Fiberoptic bronchoscopy 2 days after admission to the hospital revealed fresh blood throughout the tracheobronchial tree. The infiltrates resolved rapidly and completely during systemic steroid therapy only to reappear as the steroids were tapered, suggesting a beneficial therapeutic response. Results of serologic evaluation were negative. Transbronchial biopsies showed inflammation and hemosiderin-laden macrophages; no specific diagnosis was established. The patient was scheduled for open lung biopsy. The surgeon was concerned about the history of chest pain and requested placement of a pulmonary artery catheter, which revealed severe pulmonary hypertension. Transesophageal echocardiography and subsequent cardiac catheterization showed severe mitral regurgitation. mitral valve replacement resulted in complete elimination of symptoms.
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ranking = 813.97283358363
keywords = macrophage
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6/198. Use of high-dose corticosteroids and high-frequency oscillatory ventilation for treatment of a child with diffuse alveolar hemorrhage after bone marrow transplantation: case report and review of the literature.

    BACKGROUND: Other than relapse, pulmonary complications are the most common cause of mortality in patients who undergo bone marrow transplantation (BMT). Diffuse alveolar hemorrhage (DAH) is one noninfectious pulmonary complication of BMT. Presenting clinical findings include nonproductive cough usually without hemoptysis, dyspnea, hypoxemia, a decrease in hematocrit, and diffuse infiltrates on chest radiograph. PATIENT: We report a case of DAH after allogeneic BMT in a 6-yr-old female patient. Although a chest radiograph revealed patchy bilateral alveolar densities and large volumes of bright red blood were suctioned from the endotracheal tube, there was no evidence of coagulopathy and no infectious agent was identified on examination of bronchoalveolar lavage fluid, blood, and urine. INTERVENTION: The child was treated with high-dose corticosteroids and high-frequency oscillatory ventilation and experienced a complete clinical recovery from her pulmonary disease. RESULTS: The definition, presenting symptoms, findings and timing, and associated risk factors of DAH after BMT are reviewed. Prospective hypotheses for the pathogenesis of DAH after BMT are presented. Evidence for the role of high-dose corticosteroids for treatment of DAH after BMT and the role of high-frequency oscillatory ventilation for treatment of acute hypoxemic respiratory failure in children with diffuse alveolar disease is also reviewed. CONCLUSION: This case supports the contention that early treatment with high-dose corticosteroids is warranted in children with DAH after BMT.
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ranking = 5
keywords = bone
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7/198. Pulmonary manifestation of systemic mast cell disease.

    Systemic mast cell disease is a rare disease of unknown aetiology. Systemic infiltration and proliferation of mast cells in skin, bone marrow, gastrointestinum and lymph nodes is the central pathological feature. This study reports a patient with mastocytosis of the skin (urticaria pigmentosa) for 10 yrs. The patient was referred to hospital for dyspnoea. Chest radiograph showed moderate reticular infiltration of both lungs, computerized tomography revealed multiple lymph nodes of the mediastinum and faint nodular lesions of middle and upper areas of lungs. Transbronchial biopsy demonstrated mast cell infiltration of the lung with formation of mast cell granuloma. According to the current literature, systemic mast cell disease with pulmonary involvement is a very rare entity. After a treatment with interferon alpha-2a over 6 months, the patient's condition and particularly dyspnoea showed improvement in parallel with an amelioration of the lesions as demonstrated by thorax computed tomography.
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ranking = 1
keywords = bone
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8/198. Necrotizing pulmonary granulomata in a marijuana smoker.

    We describe the case of a heavy marijuana and tobacco smoker who presented with progressive exertional dyspnea of 2 months' duration, and bilateral nodular lung infiltrates. Examination of the lung fields was normal, and lung function tests showed mild airflow obstruction with moderately reduced gas transfer. BAL returned green-black fluid consisting predominantly of macrophages laden with carbon pigment. Thoracoscopic lung biopsy showed miliary necrotizing granulomata with an alveolar exudate of carbon-laden macrophages within macroscopically blackened lung. The differential diagnosis of pulmonary granulomata in this patient is discussed.
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ranking = 1627.9456671673
keywords = macrophage
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9/198. The role of bone scanning in the detection of metastatic calcification: a case report.

    Metastatic calcification associated with renal failure is well described. Bone scanning agents accumulate to various degrees within extraskeletal sites of metastatic calcification. The authors describe a patient with polycystic kidney disease resulting in renal failure, with the subsequent development of secondary hyperparathyroidism and metastatic calcification. Bone scintigraphy revealed abnormal uptake in both lungs, the right leg, and the right hand.
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ranking = 4
keywords = bone
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10/198. Radioisotope bone scanning in pulmonary alveolar microlithiasis.

    A 30-year-old woman was examined for a history of exertional breathlessness, swelling of her feet, and a mild dry cough of 4 to 5 months' duration. Her symptoms developed during the last month of her pregnancy, with gradually increasing dyspnea, swelling of the feet, and reduced urinary output. There was no history of expectoration, hemoptysis, chest pain, or tuberculosis. General physical examination showed no evidence of clubbing of the nails or lymphadenopathy. Chest auscultation revealed a few end-inspiratory crepitations at both lung bases. Bronchial alveolar lavage showed calcium particles, whereas results of the transbronchial lung biopsy were consistent with alveolar microlithiasis.
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ranking = 4
keywords = bone
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