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

A case of eosinophilic granuloma of the lung in a 24 years old male is presented. The initial manifestations were hemoptysis and cough. During hospitalization the bronchoscopy was performed, which revealed granuloma in the right bronchus. Computer tomography showed tumour in the right bronchus, without pulmonary changes characteristic for histiocytosis X. The granuloma was removed by bronchoscopy, but control examination performed after three months showed the residue. Once again residual tumor was removed by bronchoscopy and oral steroids were ordered. Unfortunately the next local residue was observed and the residual tumour was finally removed by thoracotomy. Now patient is free of symptoms.
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PMID:[A case of eosinophilic granuloma localized in the bronchial wall]. 1035 91

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

We report a 24-years-old woman who presented with thoracic pain after coughing. Physical exam revealed no abnormalities except pain after pressing under blade-bone area. A rib radiography and CT of the thorax showed a lonely osteolytic lesion inside eleventh left costal arch without affecting others tissues. There were no more osteolytic lesions at other levels and the histopathological study of a resection-biopsy of the lesion was diagnosed as Langerhans' cell granulomatosis. This is an uncommon disease which diagnosis can only be made through histopathological study of suspected lesions.
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PMID:[Unifocal Langerhans cell granulomatosis in a young woman]. 1110 May 38

This is a case history of a 24-year-old female patient in whom two rare pulmonary diseases occurred: Langerhans cell histiocytosis and four years later lymphangioleiomyomatosis were diagnosed. Both diseases were verified by the examination of the lung tissue removed by the surgery for pneumothorax. The patient's symptoms were characterised by coughing, dyspnoe, repeated pneumothorax. The authors summarised the characteristics and treatment of these diseases. This case history is worth of reporting for its unique rareness.
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PMID:[Rare association of rare diseases: Langerhans-cell histiocytosis and lymphangioleiomyomatosis in the lung]. 1168 Dec 32

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

Langerhans' cell histiocytosis (LCH) is a condition in which granulomas form in various tissues through the accumulation of abnormal histiocytes (Langerhans' cells), granulocytes and lymphocytes. An important distinction between LCH and primary pulmonary histiocytosis (PPH) is that smoking cessation is known to often lead to spontaneous improvement in PPH patients, while this has not been demonstrated in patients with multisystem LCH. In this case report, we describe the case of a 20-year-old man who presented with cough and palpably enlarged lymph nodes in his neck. An inguinal lymph node biopsy led to the diagnosis of multisystem LCH in the lungs, the cervical, intra-abdominal, and inguinal lymph nodes and the spleen. The patient was a smoker, and had averaged 30 cigarettes per day for 7 years. To address the multisystem involvement, intravenous pulse steroid therapy was administered (30-40 mg/kg daily) in two 3-day treatment periods separated by 3-4 weeks. The patient was also advised to stop smoking, and did so. Serial follow-up examinations confirmed an objective improvement with complete remission. The patient was still healthy at 18 months post-treatment, reflecting a dramatic and positive response to pulse steroid therapy. This encouraging result makes this case an important one to highlight.
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PMID:Effect of pulse steroid therapy in a patient with Langerhans' cell histiocytosis. 1184 29

We reviewed the clinical features of 5 cases of Langerhans' cell histiocytosis that had been referred because of pulmonary lesions. The most frequent symptom was persistent dry cough. Chest radiographs showed bilateral, symmetric reticulonodular infiltrates and accompanying cystic changes with an upper-field predominance. Pulmonary function testing (PFT) revealed moderate restrictive impairment in 3 patients. All the above features were in accordance with previous reports. The incidence of complications was, however, higher in the present cases than reported in the literature. Four cases were complicated with diabetes insipidus (DI), which caused polydipsia and polyuria on the initial presentation and was subsequently managed with intranasal desmopressin. In 4 cases, bone lesions were detected. A bone scintigram at 99mTc proved to be useful for surveillance. All patients had been followed up closely for longer than 2 years (median duration 2.8 years). Their clinical courses were generally stable without apparent decline in PFT, except that one patient with a psychiatric problem died from hypernatremia due to misuse of desmopressin. Immunosuppressive agents were given in only 2 patients including the one who died. Four transbronchial biopsies (TBB) were performed in 4 cases and at least 3 specimens were sampled from each. Histological diagnoses were made from TBB specimens in 3 patients, while the remaining 2 patients underwent an open lung biopsy. We conclude that TBB is acceptable as an initial diagnostic attempt. Multiple sampling may also contribute to preferable yield. Regarding patient management, we suggest that DI and bone lesions should be sought extensively as their incidence may be much higher than previously estimated.
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PMID:[Clinical features of five cases of pulmonary Langerhans' cell histiocytosis]. 1238 16

There exists a rarely observed association between pulmonary histiocytosis X and bronchopulmonary cancer. However, the frequency of bronchopulmonary cancer in these patients is higher than in the general population. A 28-year-old patient who currently smokes ten packs of cigarettes a year came to our department of pneumology with complains of cough and hemoptysis. An x-ray of the thorax revealed bilateral cysts and a shadow in the upper part of the right pulmonary field. In addition, a chest tomography showed multiple cysts dispersed throughout the two pulmonary fields and an irregular mass with a diameter of four centimetres in the upper right lobe. Bronchopulmonary adenocarcinoma was diagnosed during a cytologic exam of the bronchial washing. We decided to perform a thoracotomy on the patient, since there was no far metastasis. An upper lobectomy and wedge resection of the upper segment of the lower right lobe, which had been invaded by the tumour, were performed. Histology confirmed the diagnosis of adenocarcinoma. A pulmonary biopsy was carried out on the tumour-free site and showed the presence of histiocytosis X. There is a hypothesis that a neoplasm developed on the pulmonary fibrosis could be an epiphenomenon of bronchopulmonary cancer in patients who smoke and have pulmonary histiocytosis X. It is interesting to note that histiocytosis X and bronchopulmonary cancer were diagnosed at the same time, since the bronchopulmonary cancer may have occurred within a few years following the diagnosis of histiocytosis X, even if she was a smoker. Hemoptysis, which is found in 5% of patients with histiocytosis X, may suggest cancer. This young patient, a smoker, who complained of hemoptysis, is a particularly rare case of the association between pulmonary histiocytosis X and bronchopulmonary cancer whose pathogenesis is not clear cut. It is thus important to note that smoking can have major consequences, even in young people.
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PMID:[Histiocytosis X and bronchopulmonary adenocarcinoma: a rare coexistence]. 1252 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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