Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The observation concerns a patient who was professionally exposed to silica dust. Radiologically he presented a diffuse interstitial infiltrate and clinically an obstructive syndrome with emphysem. The diagnosis of silicosis was made. The development was followed during 12 years without important modification of the X-ray or the respiratory functions. The patient died of a ruptured aortic aneurysm. At the autopsy the examination of the lungs permitted to exclude the initial diagnosis of silicosis and to conclude to an histiocytosis X. This diagnostic mistake allows to get on some radiological, clinical and epidemiological aspects, characteristic or these both pathologies.
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PMID:[Histiocytosis X falsely diagnosed as silicosis]. 947 55

This review sets out to define the role of surgical biopsy in the diagnosis and prognosis of diffuse parenchymal lung disease, the pros and cons of each procedure and the interpretation of the results in the context of clinical and other investigative data. Surgical lung biopsy remains the investigation with the greatest overall diagnostic sensitivity for diffuse parenchymal lung disease. Rates of diagnosis are approximately 90-95%, compared with approximately 70% for both high-resolution computed tomography (HRCT) and transbronchial biopsy. Although open lung biopsy, and more recently videoassisted thoracoscopic surgery, have good safety records, neither technique has been utilised as frequently as might be expected, with wide regional variation for various reasons. More recently, HRCT used in conjunction with clinical and other investigative modalities, has increased the accuracy of diagnosis for some diseases, including the majority of cases of cryptogenic fibrosing alveolitis (HRCT & bronchoalveolar lavage), Langerhans' cell histiocytosis (HRCT), lymphangioleiomyomatosis (HRCT), and silicosis (HRCT). At present, the vasculitides and the idiopathic interstitial pneumonias, other than cryptogenic fibrosing alveolitis, always require a surgical biopsy. As experience with high resolution computed tomography grows, it is possible that other diseases will be able to be diagnosed without surgical biopsy. These newer modalities of investigation do have appreciable limitations and where sufficient doubt exists about diagnosis, surgical lung biopsy must continue to be utilised.
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PMID:Lung biopsy in diffuse parenchymal lung disease. 1166 2

Upper lobe fibrotic lung disease is most often associated with sarcoidosis, Langerhans cell histiocytosis, silicosis, and other pneumoconioses but is usually not associated with drug-induced lung disease. Carmustine (BCNU) is a chemotherapeutic agent known to cause pulmonary toxicity. The radiographic pattern is usually diffuse bilateral lung disease predominantly in the lung bases. Upper lobe fibrotic disease associated with BCNU has been reported to occur in children treated for central nervous system gliomas. Often the lung disease occurs years after the exposure. Despite the widespread use of BCNU in the treatment of malignancy in adults, to our knowledge, the complication of upper lobe fibrotic disease has not been reported in adults. We describe a patient who presented with pneumothorax and bilateral upper lobe pulmonary fibrosis that we believe was due to BCNU given for bone marrow transplantation as part of therapy for breast cancer. Bilateral upper lobe pulmonary fibrosis can be associated with chemotherapeutic drugs.
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PMID:Upper lobe pulmonary fibrosis associated with high-dose chemotherapy containing BCNU for bone marrow transplantation. 1274 52

Angiogenesis has been implicated in the pathogenesis of interstitial lung diseases. A correlation between serum angiogenic cytokines level of patients with idiopathic pulmonary fibrosis and radiographic manifestations or functional pulmonary changes has been described, but the role of angiogenesis in the pathogenesis of other interstitial lung diseases such as silicosis and pulmonary Langerhans cell histiocytosis remains unclear. The aim of the study was to examine the effect of sera from silicosis and pulmonary Langerhans cell histiocytosis patients on angiogenesis induced by human mononuclear cells (MNC) in relation to pulmonary function. The study population consisted of 12 patients with silicosis, 12 patients with pulmonary Langerhans cell histiocytosis (PLH), and 14 healthy volunteers. Spirometry, whole-body plethysmography, static lung compliance (Cst), and diffusing capacity of the lung for CO (DL(CO)) were performed in all patients. As an angiogenic test, leukocyte induced angiogenesis assay according to Sidky and Auerbach was used. Sera from PLH patients exerted a significant inhibitory effect on angiogenesis (P<0.001). Sera from silicosis patients significantly (P<0.001) stimulated angiogenesis compared with sera from healthy donors. However, sera from healthy donors significantly stimulated the angiogenic activity of MNC compared with the control with PBS. The mean value of DL(CO) was significantly lower in the group of patients with PLH compared with patients with silicosis (P<0.05). A significant correlation between angiogenesis index and DL(CO) was observed (P<0.05). No significant correlation between the angiogenesis index and other functional parameters was found. Sera from interstitial lung diseases patients and healthy donors constitute a source of mediators modulating angiogenesis. Sera from silicosis patients stimulate neovascularization but sera from PLH patients exert an inhibitory effect on angiogenesis. A correlation between serum angiogenic activity and DL(CO) was found.
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PMID:Angiogenic activity of sera from silicosis and pulmonary Langerhans cell histiocytosis patients in relation to lung function tests. 1921 5

Histiocytic disorders of the chest comprise a broad spectrum of diseases. The lungs may be involved in isolation or as part of systemic disease. Some of these disorders are primary and have unknown etiology, and others result from a histiocytic response to a known cause. Among primary histiocytic disorders, pulmonary Langerhans cell histiocytosis (PLCH) is the most common; others include Erdheim-Chester disease and Rosai-Dorfman disease. Adult PLCH occurs almost exclusively in adults aged 20-40 years who smoke. Pediatric PLCH is extremely rare and typically occurs as part of multisystemic disease. Erdheim-Chester disease affects middle-aged and older adults; thoracic involvement usually occurs as part of systemic disease. Rosai-Dorfman disease affects children and young adults and manifests as painless cervical lymphadenopathy. Examples of secondary histiocytic disorders are storage diseases such as Gaucher disease, Niemann-Pick disease, and Fabry disease; pneumoconiosis such as silicosis and coal workers' pneumoconiosis; and infections such as Whipple disease and malakoplakia. These disorders are characterized at histopathologic examination on the basis of infiltration of alveoli or the pulmonary interstitium by histiocytes, which are a group of cells that includes macrophages and dendritic cells. Dendritic cells are a heterogeneous group of nonphagocytic antigen-presenting immune cells. Immunohistochemical markers help to distinguish among various primary histiocytic disorders. Characteristic radiologic findings in the appropriate clinical context may obviate biopsy to establish a correct diagnosis. However, in the absence of these findings, integration of clinical, pathologic, and radiologic features is required to establish a diagnosis.
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PMID:Histiocytic disorders of the chest: imaging findings. 2576 22