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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 accuracy of high resolution computed tomography (HRCT) in the diagnosis of pulmonary lesions arising in patients with leukemia, lymphoma, or other hematopoietic proliferative disorders was evaluated in postmortem lung specimens from 35 patients with one of these neoplasms. Lungs were obtained from autopsied patients and prepared in a manner that allowed for direct pathologic-radiologic correlation. Eighty-eight pulmonary lesions with one of five radiographic patterns were identified in these 35 lungs. The gross, histologic, and radiographic changes were examined and the radiologic and pathologic diagnoses for each lesion were compared. A diffuse alveolar pattern on HRCT was primarily the result of pneumonia or intraalveolar hemorrhage, while irregular focal densities were produced by foci of hemorrhage, infarcts, lymphoma, and fungal infections. HRCT of the lung from a patient with pulmonary histiocytosis X showed a wedge-shaped subpleural density in association with a "crab-like" interstitial pattern, which was histologically shown to be an infarct with interstitial enlargement by infiltration of histiocytosis X. Air bronchograms were seen in two settings. In patients with necrotizing pneumonia, air bronchograms were associated with bronchiectasis. In contrast, air bronchograms seen in a patient with a bronchocentric lymphoproliferative disorder showed a pattern of bronchial constriction. These results suggest that HRCT may play an important role in the evaluation of pulmonary lesions in patients with leukemia, lymphoma, or other hematopoietic malignancies.
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PMID:High resolution computed tomography of inflation-fixed lungs: pathologic-radiologic correlation of pulmonary lesions in patients with leukemia, lymphoma, or other hematopoietic proliferative disorders. 154 Aug 57

A variety of diseases produces or mimics multiple, thin-walled, air-containing cavities or cysts in the lung. Although some causes of this pattern are common (bullous emphysema, multiple pneumatoceles), others are relatively rare (cystic bronchiectasis, histiocytosis X, tracheobronchial papillomatosis, and lucite ball plombage). To some extent, the radiographic features of these lesions allow their differentiation, but differential diagnosis can be difficult. This paper reviews their principal features.
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PMID:Multiple, thin-walled cystic lesions of the lung. 677 84

The authors reviewed the radiographic and computed tomographic (CT) appearances of abnormal air-filled spaces in the lung that develop in response to lung diseases. The major types of these lung diseases include infection, vessel-related or vascular-embolic disorders, bronchiectasis, emphysema, pulmonary fibrosis, adult respiratory distress syndrome and air-block diseases, and unusual disorders of the lung (such as Langerhans cell histiocytosis, Klippel-Trenaunay syndrome, and tracheolaryngeal papillomatosis). After studying the CT scans, conventional radiographs, and medical records of 150 patients with various abnormal air-filled spaces in their lungs and 300 lung specimens and the corresponding high-resolution CT scans, the authors concluded that mechanisms of air-space formation fall into five basic categories: (a) vascular occlusion or ischemic necrosis, (b) dilatation of the bronchi, (c) disruption of the elastic fiber network of the lung, (d) remodeling of the lung architecture and retractile fibrosis, and (e) multifactorial or unknown mechanisms.
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PMID:Abnormal air-filled spaces in the lung. 839 71

In order to determine the presence and distribution of Haemophilus influenzae in lung tissue sections, we obtained lung explants from 49 lung transplant recipients with cystic fibrosis (CF) (n = 16), chronic obstructive pulmonary disease (COPD) including emphysema (n = 16), bronchiectasis (n = 5), pulmonary hypertension (n = 9), Langerhans cell histiocytosis (n = 1), and idiopathic pulmonary fibrosis (n = 2). Analysis was done by selective culturing, immunoperoxidase (IP) staining, and by polymerase chain reaction (PCR). H. influenzae was cultured from specimens of the lung explants from one CF and one COPD patient. IP staining of tissue sections was positive in 24 patients (10 CF patients, eight COPD patients, two bronchiectasis patients, and four patients with noninfectious pulmonary diseases). IP-positive tissue sections were PCR-positive, and IP-negative sections were PCR-negative. H. influenzae was more frequently detected in tissue sections of lung explants from CF and COPD patients than from patients with bronchiectasis or noninfectious pulmonary diseases. H. influenzae was diffusely present in the epithelium, the submucosa of the bronchi, the bronchioles, the interstitium, and the alveolar epithelium. H. influenzae was localized extracellularly alone and in bacterial clusters, and was also associated with macrophages in CF patients. The results of this study demonstrate that H. influenzae is often present in the lungs of patients with end-stage pulmonary disease, especially CF and COPD patients. H. influenzae is diffusely present in the respiratory epithelium and subepithelial layers of the lungs of these patients.
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PMID:Haemophilus influenzae in lung explants of patients with end-stage pulmonary disease. 951 16

The aim of this pictorial essay is to illustrate the distribution of normal findings and various lung diseases on coronal reconstructions as compared with cross-sectional high-resolution CT images. The volumetric CT images were obtained at 120 kVp, 200 mA/rotation, pitch 6:1, and a high-spatial-frequency reconstruction algorithm. The scans were performed using 2.5-mm collimation and reconstructed at 1.25-mm intervals. Coronal reconstructions were obtained at 2.5-mm slice thickness and 2.5-mm intervals. The pictorial includes images of normal anatomy, emphysema, idiopathic pulmonary fibrosis, Langerhans cell histiocytosis, sarcoidosis, bronchiolitis obliterans organizing pneumonia, desquamative interstitial pneumonia, nonspecific interstitial pneumonia, lymphocytic interstitial pneumonia, pulmonary edema, tuberculosis, cytomegalovirus pneumonia, bronchiectasis, and panbronchiolitis.
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PMID:Multidetector spiral high-resolution computed tomography of the lungs: distribution of findings on coronal image reconstructions. 1236 68

Cysts and cavities are commonly encountered abnormalities on chest radiography and chest computed tomography. Occasionally, the underlying nature of the lesions can be readily apparent as in bullae associated with emphysema. Other times, cystic and cavitary lung lesions can be a diagnostic challenge. In such circumstances, distinguishing cysts (wall thickness < or = 4 mm) from cavities (wall thickness > 4 mm or a surrounding infiltrate or mass) and focal or multifocal disease from diffuse involvement facilitates the diagnostic process. Other radiological characteristics, including size, inner wall contour, nature of contents, and location, when correlated with the clinical context and tempo of the disease process provide the most helpful diagnostic clues. Focal or multifocal cystic lesions include blebs, bullae, pneumatoceles, congenital cystic lesions, traumatic lesions, and several infectious processes, including coccidioidomycosis, Pneumocystis carinii pneumonia, and hydatid disease. Malignant lesions including metastatic lesions may rarely present as cystic lesions. Focal or multifocal cavitary lesions include neoplasms such as bronchogenic carcinomas and lymphomas, many types of infections or abscesses, immunologic disorders such as Wegener granulomatosis and rheumatoid nodule, pulmonary infarct, septic embolism, progressive massive fibrosis with pneumoconiosis, lymphocytic interstitial pneumonia, localized bronchiectasis, and some congenital lesions. Diffuse involvement with cystic or cavitary lesions may be seen in pulmonary lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, honeycomb lung associated with advanced fibrosis, diffuse bronchiectasis, and, rarely, metastatic disease. High-resolution computed tomography of the chest frequently helps define morphologic features that may serve as important clues regarding the nature of cystic and cavitary lesions in the lung.
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PMID:Cystic and cavitary lung diseases: focal and diffuse. 1293 86

Bronchiolar abnormalities are relatively common and occur in a variety of clinical settings. Various histopathologic patterns of bronchiolar injury have been described and have led to confusing nomenclature with redundant and overlapping terms. Some histopathologic patterns of bronchiolar disease may be relatively unique to a specific clinical context but others are nonspecific with respect to either etiology or pathogenesis. Herein, we present a scheme separating (1) those disorders in which the bronchiolar disease is the predominant abnormality (primary bronchiolar disorders) from (2) parenchymal disorders with prominent bronchiolar involvement and (3) bronchiolar involvement in large airway diseases. Primary bronchiolar disorders include constrictive bronchiolitis (obliterative bronchiolitis, bronchiolitis obliterans), acute bronchiolitis, diffuse panbronchiolitis, respiratory bronchiolitis, mineral dust airway disease, follicular bronchiolitis, and a few other rare variants. Prominent bronchiolar involvement may be seen in several interstitial lung diseases, including hypersensitivity pneumonitis, respiratory bronchiolitis-associated interstitial lung disease, cryptogenic organizing pneumonia (idiopathic bronchiolitis obliterans organizing pneumonia), and pulmonary Langerhans' cell histiocytosis. Large airway diseases that commonly involve bronchioles include bronchiectasis, asthma, and chronic obstructive pulmonary disease. The clinical relevance of a bronchiolar lesion is best determined by identifying the underlying histopathologic pattern and assessing the correlative clinico-physiologic-radiologic context.
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PMID:Bronchiolar disorders. 1464 23

A cystic lesion in the lung is defined as a well-demarcated epithel-lined cavity, that can be mostly filled with air, water, as well as solid material content. This definition includes a wide variety of diseases such as bronchogenic cyst, abscess formation, lymphangioleiomyomatosis, Langerhans cell histiocytosis, emphysema, bronchiectasis, and pneumatoceles. Despite the difficulties in differential diagnosis, there are some diagnostic criteria for CT-scanning helping the radiologist to differentiate between these cystic entities. Moreover, clinical informations are extremely important. The most important clinical parameters include age, sex, clinical history and symptoms. Thus, a better understanding of classic CT appearance of cystic lung disease will allow more definitive diagnosis and could, in some cases, avoid biopsy.
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PMID:[Computed tomography of cystic lung diseases]. 1558 57

Smoking-related interstitial lung diseases (SRILD) are a heterogeneous group of entities of unknown cause. These diseases include desquamative interstitial pneumonia (DIP), respiratory-bronchiolitis-related interstitial lung disease (RB-ILD), pulmonary Langerhans' cell histiocytosis (LCH) and idiopathic pulmonary fibrosis (IPF). High-resolution CT is highly sensitive in the detection of abnormalities in the lung parenchyma and airways. Ground-glass attenuation can occur in DIP and RB-ILD. Whereas DIP is histologically characterized by intra-alveolar pigmented macrophages, RB-ILD shows alveolar macrophages in a patchy peribronchiolar distribution. LCH shows nodular infiltrates on histopathological examination containing varying amounts of characteristic Langerhans' histiocytes. The HRCT findings are characteristically bilateral, symmetrical and diffuse, involving the upper lobe zones with sparing of the costophrenic angles. The most prominent CT features are nodules (sometimes cavitary) measuring 1 to 10 mm in diameter, cysts and areas of ground-glass attenuation. Pathologically, IPF is characterized by its heterogeneity with areas of normal lung, alveolitis and end-stage fibrosis shown in the same biopsy specimen. High-resolution CT findings consist of honeycombing, traction bronchiectasis and intralobular interstitial thickening with subpleural and lower lung predominance. Since coexisting lesions in the same cases have been observed, a better understanding of the different smoking-related interstitial lung diseases (SRILD) allows a more confident and specific diagnosis.
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PMID:Smoking-related interstitial lung diseases: radiologic-pathologic correlation. 1686 68

An 80-year-old nonsmoking man was referred to our hospital with bilateral perihilar pulmonary opacities. He had a history of epilepsy, sclerosing cholangitis, cutaneous lesions previously diagnosed as localised Langerhans cell histiocytosis. Symptoms included dry cough and dyspnea. Chest CT showed bilateral perihilar alveolar consolidation with bronchiectasis. Histological examination of a lung biopsy showed typical features of Langerhans cell granulomatosis. Investigations revealed anterior and posterior hypopituitarism. An important improvement occurred with corticosteroid and vinblastine treatment.
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PMID:[Unusual pulmonary presentation of systemic Langerhans cell histiocytosis]. 1828 Nov 29


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