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

Spontaneous pneumothorax (SP) is commonly encountered by general surgeons and is usually caused by rupture of subpleural blebs. Although uncommon, SP may be due to primary pulmonary histiocytosis X, a disease characterized by eosinophilic and histiocytic infiltration of lung tissue. It primarily affects young smokers and usually presents with symptoms of cough, dyspnea, and chest pain. A young adult male with this disease presented with recurrent SP and was successfully treated with thoracoscopic stapling of bullae and pleural abrasion.
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PMID:Recurrent spontaneous pneumothorax in pulmonary histiocytosis X. 979 65

Erdheim-Chester disease is a clinicopathologic entity defined by a characteristic pattern of symmetric osteosclerosis caused by an infiltrate of mononuclear cells that include prominent numbers of foamy histiocytes. About half of patients have extraskeletal manifestations, including involvement of the hypothalamus/posterior pituitary, orbit, retroperitoneum, skin, lung, and heart. Pulmonary involvement is an uncommon but important manifestation of Erdheim-Chester disease because it causes significant morbidity and mortality. A review of the Mayo Clinic files produced four patients with confirmed Erdheim-Chester disease in whom lung biopsy had been performed. One additional patient was included from the University of Pittsburgh. Four patients were women. The mean age was 53.6 years (range 25-70 years). All patients had bilateral and symmetric sclerotic bone lesions characteristic of Erdheim-Chester disease, although in three the skeletal abnormalities were discovered only after lung biopsy. Four patients had dyspnea, and one also had a dry cough. One patient died 17 months after diagnosis. Chest radiographs showed diffuse interstitial infiltrates in all patients, with an upper zone predominance in three. Thoracic computed tomography (CT) scans showed thickening of the visceral pleura and interlobular septa with patchy associated fine reticular and centrilobular opacities and ground glass attenuation. Lung biopsy specimens showed an infiltrate of foamy histiocytes, lymphocytes, and scattered Touton giant cells with associated fibrosis in a striking lymphatic distribution. The infiltrate involved visceral pleura, interlobular septa, and bronchovascular bundles. Immunohistochemical stains were positive for CD68 in all cases and S-100 protein in four cases. Stains for CD1a were consistently negative. Ultrastructural studies in one case showed no Birbeck granules. Although in bone the histologic features of Erdheim-Chester disease may overlap with Langerhans' cell histiocytosis, its expression in the lung is distinct. Lung involvement in Erdheim-Chester disease has emerged as a unique radiographic and histologic entity.
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PMID:Erdheim-Chester disease: clinical, radiologic, and histopathologic findings in five patients with interstitial lung disease. 988

Xanthoma disseminatum (XD) is a rare mucocutaneous xanthomatosis classified as a benign form of non-Langerhans' cell histiocytosis. The case history is presented of a 61 year old woman with XD who developed dyspnoea and spirometric features of airflow obstruction. Bronchoscopy and computed tomography confirmed involvement of the large and medium sized bronchi and she subsequently died from acute respiratory failure. The post-mortem findings and the importance of respiratory tract disease in this unusual condition are discussed.
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PMID:Xanthoma disseminatum with respiratory tract involvement and fatal outcome. 1063 38

Histiocytosis X (HX), also referred as Langerhans cell granulomatosis is a disorder characterized by the presence of destructive granulomas containing Langerhans cells, lymphocytes, eosinophils and fibroblastes in the involved organs. Three presentations are commonly observed: 1) nonproductive cough or effort dyspnea, 2) spontaneous pneumothorax 3) incidental pulmonary infiltrates on chest X-ray in asymptomatic patients. HRCT may be helpful in the initial diagnosis of pulmonary HX. HRCT scans show nodules, cysts and estimate the extent of disease. But the final diagnosis of histiocytosis X requires the histologic demonstration of specific histiocytosis X cell in biopsy specimens of the lung. The aim of this study was to define the importance of the detection of Langerhans cells in bronchoalveolar lavage fluid (BALF) for the diagnosis of HX. The searched cells express a specific CD1 antigen, recognized by the monoclonal antibody OKT-6. In our study the demonstration of more than 5% of CD1 positive cells was defined to confirm HX. We have studied the BALF in 21 patients with suspected histiocytosis X. In BALF of 4 patients more than 5% of CD1 positive cells were found. In 1 of them HX was confirmed with open lung biopsy. Two patients displayed 5% of CD1 positive cells. The final diagnosis of the first patient was hypersensitivity pneumonitis and of the second one was bronchitis chronica. In 5 patients out of 15 patients in whom less than 5% of CD1 positive cells were found histiocytosis X was histologically proven. In other 10 patients the following disorders were histologically recognised: pulmonary emphysema 3 cases, pneumoconiosis-3, LMA-BOOP-1, sarcoidosis-1 and pleuritis eosinophilica-1. The estimation of Langerhans cells in BALF can be a useful method among the diagnostic procedures for histiocytosis X. It is necessary to remember that demonstration of less than 5% of CD1 positive cells do not exclude histiocytosis X.
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PMID:[Usefulness of CD1 expression on surfaces of cells in bronchoalveolar fluid for diagnosis of histiocytosis X--our experience]. 1064 82

Pulmonary Langerhans' cell histiocytosis and eosinophilic granuloma are the terms used to describe a Langerhans' cell granulomatous interstitial lung disease of unknown aetiology, occurring predominantly in smokers and involving primarily lungs, bones, skin and lymph nodes. In this report a patient with fever, fatigue, dyspnoea, nocturnal perspiration and thoracic pain is described. The high-resolution computed tomography of the chest and histological examination of lung biopsies suggested the diagnosis of pulmonary Langerhans' cell histiocytosis. The disease was limited to the lung, since further investigations did not show any other localization. The patient had a good clinical outcome with avoidance of smoking and steroid therapy. The computed tomography scan follow-up showed a partial resolution of pulmonary lesions.
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PMID:Favourable outcome of a case of pulmonary Langerhans' cell histiocytosis. 1078 15

Erdheim-Chester disease (ECD) is a rare non-Langerhans' cell histiocytosis that may present with pulmonary symptoms. The condition seems to be nonfamilial and typically affects middle-aged adults. Radiographic and pathologic changes in the long bones are diagnostic, but patients often present with extraskeletal manifestations. Advanced pulmonary lesions are associated with extensive fibrosis that may lead to cardiorespiratory failure. The clinical, radiologic, and pathologic features of six patients with ECD with lung involvement are presented. The patients were three men and three women (mean age, 57). Five presented with progressive dyspnea, and one presented with diabetes insipidus. Open-lung biopsies showed histiocytic infiltrates in a lymphangitic pattern with associated fibrosis and lymphoplasmacytic inflammatory infiltrates. The histiocytes did not stain with periodic acid-Schiff. Immunoperoxidase studies performed on specimens from five of six patients showed that the histiocytes were positive for CD68 and Factor XIIIa and negative for CD1a. Specimens from two patients exhibited immunoreactivity for S-100 protein. Electron microscopy studies performed on specimens from two patients showed phagocytic lysosomes but no Birbeck granules. Clinical follow-up of up to 16 years was available. At the end of that time, five patients were dead of complications related to their disease; one patient remains alive 4 years after diagnosis but with severe respiratory compromise. ECD is a rare non-Langerhans' cell histiocytosis that may present as interstitial lung disease and resemble other pulmonary conditions, particularly usual interstitial pneumonitis and pulmonary Langerhans' cell histiocytosis. Recognition of this entity will allow better assessment of its true incidence, therapeutic options, and prognosis.
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PMID:Pulmonary pathology of Erdheim-Chester disease. 1091 34

Interstitial lung diseases (also known as diffuse infiltrative lung diseases) are a heterogeneous group of parenchymal lung disorders of known or unknown cause. These disorders are usually associated with dyspnoea, diffuse lung infiltrates, and impaired gas exchange. The majority of interstitial lung diseases are of unknown cause. Known causes of interstitial lung disease include inhalation of organic and inorganic dusts as well as gases or fumes, drugs, radiation, and infections. This review summarizes the clinical, radiological, and histopathological features of four interstitial lung disorders that have been linked to smoking. These disorders include desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, pulmonary Langerhans' cell histiocytosis, and idiopathic pulmonary fibrosis. Available evidence suggests most cases of desquamative interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, and pulmonary Langerhans' cell histiocytosis are caused by cigarette smoking in susceptible individuals. Smoking cessation should be a main component in the initial therapeutic approach to smokers with these interstitial lung diseases. In addition, smoking appears to be a risk factor for the development of idiopathic pulmonary fibrosis.
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PMID:Smoking-related interstitial lung diseases: a concise review. 1130 41

Langerhans cell histiocytosis with only pulmonary involvement is a rare, probably reactive, proliferating disease of young and smoking persons. During last 7 years 7 cases of Langerhans cell histiocytosis were diagnosed in our Department. There were 6 men and one woman. Mean age was about 32 years. All of them were smokers. Intensity and duration of smoking varied widely from 5 to 40 cigarettes/day and 5 to 30 years respectively. The most frequent symptoms were dry cough, dyspnea. Two patients had no complaints. Pneumothorax preceded diagnosis in 4 patients. Duration of symptoms varied from 3 months to 10 years--in a patient who was previously diagnosed as allergic alveolitis. All patients had typical radiological and HRCT appearance of disseminated nodular lesions with small cysts formation with sparing of costo-phrenic angles. Three of patients had very advanced disease with cystic lung destruction. Restriction was noticed in those three patients. Diminished values of compliance and diffusing capacity for CO was observed in four patients. Bronchoscopy examination of bronchial tree revealed only slight inflammation in all patients. BAL was assessed for presence of CD1a cells in 6 cases, but in no one patient elevated number of those cells was noticed (2-4.5%). In all patients open lung biopsy was performed, and typical histologic appearance was observed. In six patients also CD1a positive cells were revealed in lung specimens.
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PMID:[Pulmonary Langerhans cell histiocytosis--clinicopathologic analysis of 7 cases]. 1173 89

A 42-yr-old woman was referred to our hospital because of multiple small nodules in a chest radiograph. She had no symptoms such as dyspnea, cough or sputum. A chest CT revealed many centrilobular small nodules and thin-walled cysts with predominance in the peripheral area of the lungs. The specimens obtained by thoracoscopic surgery showed granulomas with scattered eosinophils and numerous Langerhans' cells. The Langerhans' cells were positive for both S-100 protein and CD1a. These findings are compatible with pulmonary Langerhans' cell histiocytosis (LCH). Since the granulomas showed no fibrotic changes, the LCH may have been in its early stages. However, there were clusters of lymphocytes and macrophages around the terminal and respiratory bronchioles, and cystic lesions without cellular infiltrates, in the specimens. The former histologic findings suggested respiratory bronchiolitis causing interstitial lung disease and the latter are indistinguishable from centrilobular emphysema. Therefore, these smoking-related diseases may have been superimposed on the LCH in this patient.
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PMID:[A case of pulmonary Langerhans' cell histiocytosis]. 1453 8

Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of an unknown cause affecting women of reproductive age and characterised by smooth muscle proliferation along lung lymphatic channels. Pneumothorax develops in up to 80% of patients with LAM and may be the presenting manifestation of the disease. Pneumothorax also precedes or complicates the clinical course of 25% of patients with Langerhans'-cell histiocytosis (LCH, histiocytosis X) pathologically characterised by involved tissue infiltration with large numbers of unusual Langerhans' cells, often organised as granulomas. A 41-year-old female patient was treated twice by simple tube drainage due to left pneumothorax in 1996. She was then diagnosed with chronic obstructive lung disease demonstrating with dyspnea, cough and wheeze. Abnormalities found in the high-resolution computed tomography (HRCT) scanning were characteristic of LAM with thin-walled parenchymal cysts distributed homogeneously in both lungs and with thickening of interlobular septa. A 38-year-old man was hospitalised due to chronic lung failure in the course of LCH characterised by small, poorly limited nodular lesions and thin-walled cysts revealed in HRCT scans. For two years of observation, he has suffered five episodes of right pneumothorax. When diagnosing pneumothorax in the middle-age patients, the possible cause of the disease is LAM or LCH. The use of HRCT scanning may enable good determination of the nature and distribution of parenchymal abnormalities found in the diseases.
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PMID:Difficulties in the diagnosis of rare immunological diseases manifesting with cystic lung disease and spontaneous pneumothorax: Case reports. 1498 35


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