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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Needle biopsy was performed in 100 children with diffuse interstitial pneumopathy. Severe histological lesions were noted in the biopsy sections in 4 out 5 cases. Those most frequently observed (57 cases) were non-specific lesions of diffuse interstitial pneumopathy (DIP) or idiopathic fibrosis, the course of which was followed, in some cases, by repeating the biopsy. Specific lesions, especially idiopathic pulmonary hemosiderosis (8 cases), were noted in 22 children, either associated with interstitial fibrosis or apparently better tolerated. The other 16 cases included the lipoidoses (Niemann-Pick, histiocytosis X), alveolar microlithiasis, and some specific inflammatory diseases (tuberculosis, syphilis) or those related to inhalation of a foreign body.
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PMID:[Results of lung biopsy in interstitial pneumopathies in children. A report on 100 cases (author's transl)]. 54 6

High resolution computed tomography (TDM-HR) is now the technique of choice in the diagnosis and management of diffuse infiltrative lung disease (PID). After a brief review of the technique the authors describe the normal appearance; anatomical observations and the in vivo findings have shown that TDM-HR allow for the exploration of details of structure down to the second pulmonary lobule. Thus, through the alterations that are transmitted in the lobular area, and from its contents and its limits, PID has led to the elaboration of a new semeiology. The authors review the basic computed tomographic images and correlate these in each case with the histological evidence. The spatial distribution and the time sequence of the elementary images are the two other terms in the diagnostic equation of PID. The spatial distribution of several elementary images presents in TDM-HR a superior aetiological pointer to that which is furnished by thoracic radiographs; and the time sequence may furnish a useful indication as to the progress of the treated disease. Sarcoidosis, histiocytosis X, idiopathic interstitial fibrosis and lymphangitis carcinomatosis would serve as examples. Nevertheless, the authors point out that it would be dangerous during the period of evaluation to prematurely extend to all cases of PID conclusions which are only possible to make at present in a restricted number of disorders.
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PMID:[Diffuse infiltrative lung diseases: histological support of elementary lesions observed on tomodensitometry]. 150 84

In Langerhans' cell histiocytosis, the prognostic significance of pulmonary disease is controversial. The clinical and radiological features and lung function tests of Langerhans' cell histiocytosis patients presenting to a single tertiary referral center between 1981 and 1987 were reviewed. Age at diagnosis ranged from 2 weeks to 16 years (median 1.7 years) and the male-female ratio was 2.4:1. No child presented with lung involvement alone. In 18 (40%) of 45 patients with multisystem disease there was clinical and/or radiological evidence of lung pathology. Another 6 children (13%) with normal chest roentgenograms had abnormal lung function tests, suggesting subclinical ("occult") involvement. Those with overt lung disease tended to present at a younger than average age (median 0.6 years). The most common functional disturbance was reduced lung or respiratory compliance with reduced lung volumes. Patients with and without lung involvement showed a similar pattern of involvement of other organs, with skin and bone most commonly affected. Of the 45 children with multisystem disease, 38 (84%) survived 2 to 7 years after diagnosis; there was a similar proportion of deaths in children with and without lung involvement. It is concluded that lung involvement occurs in nearly half of young children with multisystem Langerhans' cell histiocytosis but does not adversely affect outcome.
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PMID:Lung involvement in Langerhans' cell histiocytosis: prevalence, clinical features, and outcome. 174 Dec 22

The main criteria for the morphologic diagnosis of fibrosing lung disease are the type of inflammation, the localization of the lesion within the lung, and the microtopographic pattern of inflammation and fibrosis. Among the microtopographic patterns six types (perilobular, intraalveolar, alveoloseptal, bronchiolitic, vasculitic, bronchiolectatic), all corresponding to pathogenetic mechanisms can be recognized. Mainly one of these basic patterns is realized in sarcoidosis, histiocytosis X and shock lung. In other diseases a combination of two or more patterns may occur. The microtopographic pattern can be shown best in open biopsies after unfolding of the lung tissue. The findings in bronchoalveolar lavage do not reflect the real composition of the inflammatory infiltration of the lung tissue, and especially T-lymphocytes are usually overrepresented.
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PMID:[Contribution of pathology to diagnosis of fibrosing lung diseases]. 209 8

To evaluate the usefulness of anti-T6 monoclonal antibody cell analysis in the assessment of diffuse lung disease, 77 bronchoalveolar lavages (BAL) were performed on 70 subjects: 18 normal smokers, 14 normal nonsmokers, 30 patients with chronic interstitial lung diseases (15 sarcoidosis, 12 idiopathic or associated pulmonary fibrosis, 3 histiocytosis X) and 8 patients with diffuse lung neoplastic disorders. The percentage of T6-positive cells was significantly higher in normal smokers than in normal nonsmokers (p less than 0.05). Positive T6 cells were absent or less than 1% in normal subjects, in patients with interstitial lung diseases and in patients with diffuse lung cancer, except in a case of desquamative interstitial pneumonitis, who had 2% of reacting cells. In contrast, such cells were always 3% or higher in the 6 BAL performed in histiocytosis X patients (p less than 0.05).
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PMID:Bronchoalveolar lavage analysis with anti-T6 monoclonal antibody in the evaluation of diffuse lung diseases. 263 45

Thoracoscopy was performed under local anesthesia in 419 patients suffering from a diffuse lung disease. In 85% of the cases diagnosis was clarified by thoracoscopy. All other cases were confirmed by means of an open lung biopsy. The best results were obtained in sarcoidosis of the stages II and III, the sensitivity being 0.98. Tumour-conditioned diffuse lung diseases were clarified in 88% of the cases; proof of an interstitial pulmonary fibrosis or interstitial pneumonia was established in 85% of the patients. Results regarding histiocytosis X were poor: thoracoscopic-bioptic proof was successful in only 42% of the patients. In 419 examinations we only detected a severe complication (air embolism). Drainage times were on the average between 4 and 5 days. On the whole, the method was characterised in the field of diagnosis of diffuse lung diseases by a high degree of sensitivity and satisfactory specificity. Both in respect of the invasiveness of the examination and its sensitivity it occupies an intermediate position between peripheral bronchoscopically obtained biopsy and surgical open lung biopsy, representing a valuable extension of the diagnostic instrumentarium if the indication is carefully considered.
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PMID:[Thoracoscopy in diffuse lung diseases]. 271 51

We cared for 124 pediatric patients with a histologic diagnosis of Langerhans' cell histiocytosis (histiocytosis X) over a period of 14 years. Clinical, laboratory, and radiographic findings were analyzed. The most frequent manifestations were bone lesions, lymph node involvement, and skin infiltration. Liver disease was noted in 50% of patients and lung disease in 23%; hematologic changes were also frequent. Dysfunction and involvement of these three organ systems, plus age of onset, distinguished the group of patients with the highest mortality. All patients with generalized disease or organ dysfunction were treated with systemic chemotherapy. The actuarial survival curve at 10 years was 63%.
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PMID:Langerhans cell histiocytosis: clinical experience with 124 patients. 326 10

Langerhans' cells are a defined subpopulation of the mononuclear phagocyte system known to accumulate in the lung in histiocytosis X, an interstitial lung disorder strongly linked to cigarette smoking. To evaluate the hypothesis that cigarette smoking itself may be associated with the accumulation of Langerhans' cells in the lung, normal nonsmokers (n = 5) and normal smokers (n = 10) were evaluated by bronchoalveolar lavage for the presence of Langerhans' cells as identified by the OKT6 monoclonal antibody and by transmission electron microscopy. While the OKT6 antibody identified 0.1 +/- 0.1% of the cells recovered from nonsmokers, it labeled 1.1 +/- 0.3% of those recovered from smokers (p less than 0.01). Furthermore, while electron microscopy demonstrated no Langerhans' cells among the lavage cells from nonsmokers, 0.4 +/- 0.1% of the cells recovered from normal smokers contained characteristic intracytoplasmic Birbeck granules, positively identifying them as Langerhans' cells. We conclude that cigarette smoking is associated with an expansion in the population of Langerhans' cells on the epithelial surface of the lower respiratory tract. While the mechanisms underlying this accumulation are unknown, it is possible that the properties of these cells contribute to the derangements of the pulmonary parenchyma found in cigarette smoking and establish a biologic link to the already observed epidemiologic association between histiocytosis X and cigarette smoking.
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PMID:Accumulation of Langerhans' cells on the epithelial surface of the lower respiratory tract in normal subjects in association with cigarette smoking. 327 1

Bronchoalveolar lavage has emerged as a useful technique for the study of pulmonary interstitial disorders. Several types of information are provided by the evaluation of lavage fluid. First, the identification of cellular constituents helps to separate inflammatory processes in which lymphocytes predominate (for example, sarcoidosis, hypersensitivity pneumonitis, and berylliosis) from those in which neutrophils or macrophages predominate (for example, idiopathic pulmonary fibrosis and histiocytosis X). Second, the cells removed during lavage can be studied for their immune properties and function; tested with specific antigens, in diseases such as berylliosis and hypersensitivity pneumonitis; and examined for the presence of unique surface antigens with monoclonal antibodies (for example, histiocytosis X). Third, in conjunction with scanning electron microscopy and electron probe analysis, lavage makes possible the identification of inorganic particles in alveolar macrophages of patients with pneumoconiotic lung disease. Finally, although lavage is still an investigative procedure for most pulmonary disorders, it has an established role in the diagnosis of opportunistic infections in the immunocompromised patient.
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PMID:Bronchoalveolar lavage: role in the pathogenesis, diagnosis, and management of interstitial lung disease. 388 Oct 71

Respiratory tract fluid, produced from an enormous area spanning the mucosa of the nose to the alveolar surface, is a complex mixture of serum transudate and locally secreted proteins and glycomucoproteins and of inflammatory and immune effector cells intermingled. Its analysis is important in understanding the pathogenesis of respiratory diseases and remains essential for the clinical diagnosis of most lung disorders. Many basic facts about the formation and composition of this fluid remain unknown, and little information exists about absorptive mechanisms along the airways. Respiratory fluid is not homogeneous but has unique regional characteristics that are becoming better appreciated as more selective sampling methods are devised. Above all, it is a dynamic substance in healthy airways and diseased ones, and any specimen is just a point-in-time sample that can change in composition, often making serial analysis and comparisons necessary. Nasal fluids currently have limited diagnostic application except in allergic rhinitis. Expectorant (sputum) telescopes fluid and cells from all areas and is not from a specific locale, so the trend is to retrieve more selective and regional specimens of airways fluids. Technology largely does not exist to collect area specimens, except for bronchoalveolar lavage, which generally samples the alveolar surface. Clearly, bronchoalveolar lavage fluid analysis has been the favored way to characterize the peripheral air-spaces for the past 10 years or so, and most of this monograph has been devoted to normal data derived from lavage specimens and to a few examples of lung disease that reflect this burgeoning application. In many respects, results obtained from lavage fluid are virtually in catalogue form at present, and it remains to the future to know how some of the observations will help make diagnosis better or elucidate pathogenic mechanisms. Generally, bronchoalveolar lavage fluid analysis has led to better concepts of immunopathology of many diseases and provided new ways to monitor the evolution of certain diseases, especially the diffuse interstitial lung disorders, but development of specific criteria for diagnosis has been less rewarding. However, certain patterns of lymphocyte-predominant alveolitis, suggesting sarcoidosis or hypersensitivity diseases, recognition of specific T lymphocyte defects and opportunistic microorganisms as in AIDS, and the use of microprobe electron analysis to identify cellular particulates all point to more precision of diagnosis. Alveolar proteinosis and histiocytosis X may be detected from lavage fluid components. The prospects are truly exciting.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Respiratory tract fluids: analysis of content and contemporary use in understanding lung diseases. 636 22


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