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

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.
Rev Mal Respir 1992
PMID:[Diffuse infiltrative lung diseases: histological support of elementary lesions observed on tomodensitometry]. 150 84

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.
Rev Mal Respir 1992
PMID:[Pulmonary lesions in Wegener's disease. Report of the French Anatomo-clinical Research Group. Study of 40 pulmonary biopsies]. 150 87

Open lung biopsy enables a diagnosis to be made in cases of diffuse infiltrative pulmonary disease. It was developed to aid the diagnosis of opportunistic infectious disease observed in virus infections in immune deficiency states and during transplants. Open lung biopsy involves the partial resection of the lung parenchyma. The availability of the mechanical stapler has resolved the problem of parenchymal air leaks when suturing lung tissue. Open lung biopsy should be carried out when endoscopic techniques have failed to achieve a diagnosis, including bronchoalveolar lavage and transbronchial biopsy. The place of open biopsy continues to develop. It is becoming less important when other techniques have become available for certain diagnoses such as Pneumocystis carinii, histiocytosis X, alveolar proteinosis and the rejection of lung transplants.... It should be carried out early when a particular diagnosis might change the management of a patient.
Rev Mal Respir 1992
PMID:[Surgical lung biopsy]. 154 45

Histiocytosis is a rare cause of diffuse interstitial pneumonia. Its aetiology is not known and the outcome is often unsatisfactory. In 10 subjects in whom the diagnosis of histiocytosis X was established at CHU in Grenoble, 7 could be followed up. We report the clinical, functional, radiological and high resolution tomographic data on these 7 subjects suffering from histiocytosis X and followed up for a period between 3 and 13 years (8 +/- 3 years). All the subjects were symptomatic at the time of diagnosis. The clinical outcome amongst the 10 subjects included 2 patients who died, 2 who stabilised, 1 worsened and there were 4 clinical cures, 1 patient was lost to follow up. The respiratory function tests of the 7 subjects who were followed up was characterised by the appearance of airflow obstruction and a significant fall in the DLCO/VA (KCO) of 68 +/- 17% of the predicted value to 43 +/- 13%. An analysis comparing the initial pulmonary radiography to the current films showed that the reticular nodular lesions tended to progress to a diminution in size, while there was an increase in the reticulation. The high resolution computed tomography was confirmed as better technique than pulmonary radiography in detailing the cystic and nodular lesions. This was the only examination which correlated with DLCO/VA (R: 0.8; p: 0.018). A progressive model for computed tomographic lesions is proposed. Computed tomography appears to be the key examination, orientating the diagnosis based on the association of the nodules and cysts and enabling a better appreciation of the severity of the pulmonary disease taking account of its excellent correlation with diffusion.
Rev Mal Respir 1992
PMID:[Value of high resolution tomodensitometry in pulmonary histiocytosis X. Radiological, clinical and functional correlations]. 161 4

The pulmonary primary localization of the histiocytosis X is rather rare. We describe here a case of histiocytosis X with a localization exclusively at pulmonary level in a 38 years old young man who is a heavy smoker. A dry cough, asthenia and an increasing dyspnoea were the first symptoms of the disease. The radiological picture showed a widespread pulmonary intersticepathy. The diagnosis has been obtained testing some fragments of the pulmonary parenchyma taken through a diagnostic thoracotomy with an electron microscope.
Arch Monaldi Mal Torace
PMID:[A case of primary pulmonary histiocytosis X]. 215 51

This is a case report of a 39 year old patient in whom histiocytosis X was discovered following a cough which had appeared several months before. The radiological investigations were distinctive immediately associating the alveolar lesions and the excavated macronodules. The clinical examination was normal but there were some laboratory features suggesting inflammation. The diagnosis was achieved following a surgical biopsy which on histological examination showed histiocytic granulomas and the immunology revealed cells which were positive for OK T6 monoclonal antibodies and S100 antiprotein antibodies. There was subsequently a spontaneous regression with disappearance of the nodular shadows only leaving a few cystic forms. This observation is distinctive and is characterised by the rare appearance of the initial radiology and also confirms the predisposing factor of smoking in the pathogenesis of histiocytosis X.
Rev Mal Respir 1990
PMID:[Histiocytosis X: excavated macronodular form. Initial alveolar phase with spontaneous regression. Role of smoking]. 227 Mar 49

The authors report 11 cases of bone lesions associated with histiocytosis X in adults, with a total of 25 bone sites: 22 were detected on X-rays and 3 were detected on scintigraphy when the corresponding X-rays were normal. They stress the high incidence of cranio-facial involvement, predominantly in the maxillae, with premature localised loosening of the teeth. They recommend X-rays in the staging of histiocytosis X and reserve scintigraphy for the cases with normal or doubtful X-rays. The bone lesions do not affect the prognosis of histiocytosis X, which depends on the associated visceral lesions. It generally has a favourable outcome and requires careful treatment: limited to a localised procedure for the purposes of histological diagnosis or to control the local underlying visceral risk or the functional complications. Bone lesions do not usually participate in the decision to treat histiocytosis X by chemotherapy.
Rev Rhum Mal Osteoartic 1985 Oct
PMID:[Bone involvement in histiocytosis X in adults. 11 cases]. 387 68

The anatomical lesions of three subjects suffering from pulmonary histiocytosis X and treated with lung transplantation were studied in order to detail the changes occurring in late stages of the disease; the early stages are known on account of lung biopsies done with a diagnostic aim. All the subjects were, at the time of transplantation, in a precarious clinical state and were practically bed-bound as a result of their dyspnoea with a history of histiocytosis X which had been known for 9, 7 and 3 years respectively; one patient had a cardiopulmonary bypass and two unilateral lung transplant. The results of the histological study produced data which was slightly discordant from the communly held ideas; the de Langerhans cells persist in large numbers and after many years of the evolution of the disease, even though other elements of the granuloma have practically disappeared. Fibrosis is absent playing no role in the genesis of the respiratory failure. The lesions are destructive, leading to a disappearance of the alveolar capillary bed which is the source of the dyspnea. The honeycomb cavities to the destroyed lung are varied in nature: most correspond to the disappearance of the dividing walls of the alveoli or the pulmonary acinus, some others related to dilated terminal bronchioles and finally others to hollowed out granulomas which have become cavities. The peculiar characteristic of the lung allows for destructive cavities to be produced by the airflow, submitted to a process of auto-aggravation by the elastic forces and the tension of the pulmonary parenchyma.
Rev Mal Respir 1993
PMID:[Severe late stage lesions of pulmonary histiocytosis X. Report of 3 transplantations]. 834 66

Pulmonary Langerhans cell histiocytosis (LCH) is an uncommon disorder occurring most often in young smokers. Histologically, the disease is characterized by granulomatous lesions containing LC that destroy distal bronchioles. The etiology of the disease remains unknown, but progress has been made in understanding its pathogenesis. Modifications in the bronchiolar epithelium induced by smoking, such as the increased secretion of GM-CSF by these cells, are probably responsible for the initial accumulation of large numbers of LC. However, given the rarity of pulmonary LCH compared with the frequency of smoking, an as yet unidentified genetic predisposition may also be necessary for the development of the disease. Although LC in LCH granulomas may be clonal in origin, several observations argue against the idea that the disease, which can regress spontaneously, is a malignant process. Cells of dendritic cell lineage (including LC), are potent antigen presenting cells, suggesting that pulmonary LCH results from an uncontrolled immune response initiated by LC. Consistent with this idea, LC and T-cells are the predominant cell populations found in the early lesions of pulmonary LCH, and unlike LC in the normal bronchial mucosa and those accumulating in other pathologic situations, LC in pulmonary LCH granulomas express surface molecules important for the activation of T-lymphocytes. A number of mediators are produced in the microenvironment of granulomas that probably influence the outcome of the local immune and inflammatory reaction. Ultimately, precise knowledge of the pathogenesis of this disorder should permit the development of specific treatment. In the interim, therapies aimed at modifying the state of activation of LC in the granulomatous lesions may prove useful.
Rev Mal Respir 1999 Apr
PMID:[Pathogenesis of adult pulmonary Langerhans-cell histiocytosis]. 1033 58

A role for viruses in the development or course of the main idiopathic chronic infiltrative lung disease has been suggested for a long time. Viruses that have usually been incriminated in cryptogenic fibrosing alveolitis are hepatitis C virus, whose role has not been accurately proven, and latent viruses including Epstein-Barr virus and adenovirus. These latent viruses might be re-activated in the lung of patients immunocompromised by treatments and might be accountable for disease progression. However, published studies have been very conflicting and the only clinical trial testing ribavirin has failed to demonstrate a beneficial effect. In sarcoidosis, the responsibility of human herpesvirus 6 and 8 and retroviruses has not been proven. Finally, data in the literature do not support a link between Langerhans cell histiocytosis and human herpesvirus 6 and 8. These viruses may act by several mechanisms: viral proteins may be antigens driving an appropriate immune response; they may also behave as transactivating factors to control the expression of various genes involved in immune response, cell growth or synthesis of matrix proteins.
Rev Mal Respir 2001 Jun
PMID:[Chronic infiltative lung disease and viruses]. 1146 86


1 2 Next >>