Gene/Protein Disease Symptom Drug Enzyme Compound
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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 17-year-old adolescent was admitted to Oita University Hospital with non-productive cough and exertional dyspnea. She had been smoking approximately 10 cigarettes per day for two years. When the patient was three years old, she underwent surgical removal of skull tumor of Langerhans cell histiocytosis. Initial chest CT scans showed coalescing thick-walled air cysts surrounded by micronodules in both lungs, most predominantly in the middle and upper lung fields. Bronchoalveolar lavage fluid contained 2.3% of CD1a-positive cells and video-assisted thoracoscopic lung biopsy disclosed granulomatous lesions consisting of histiocytic cells containing S-100 protein but without CD68 antigen allowing a diagnosis of pulmonary Langerhans cell histiocytosis. She stopped smoking, resulting in spontaneous resolution of the coalescing air cysts which were replaced by funicular scarring within two years. In case of extra-pulmonary Langerhans cell histiocytosis in children, the close relationship between cigarette smoking and pulmonary involvement should be informed to the parents to prevent the patient starting smoking in the future.
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PMID:[A case of pulmonary langerhans cell histiocytosis presenting disappearance of coalescing air wall cysts after smoking cessation]. 1605 Apr 71

Early-onset chronic obstructive pulmonary disease (COPD) is designated as onset under age 50. We report a case of early-onset COPD with recurrent pneumothorax. A 29-year-old woman visited our hospital with productive cough and dyspnea on exertion. CT scan of the chest demonstrated severe panlobular emphysema. A pulmonary function test showed a reduction in FEV1.0 (41% of the predicted value). A diagnosis of severe COPD was made. Her symptoms and pulmonary function improved after the treatment of inhaled corticosteroid, long-acting beta2-agonist, and anti-cholinergic drugs. She had pneumothorax at least 8 times in the right lung. The level of alpha1-antitrypsin was normal. On the basis of the characteristics of the appearance of the chest X-ray and CT scan, the possibility of bronchiolitis obliterans, lymphoangioleiomyomatosis or Langerhans cell histiocytosis was thought to be low. We considered that several factors, such as high susceptibility, pulmonary infection during her childhood, bronchial asthma, malnutrition, smoking history from an early age, and long-term passive exposure to cigarette smoke may have contributed to the development of early-onset COPD in the present case.
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PMID:[A case of early-onset COPD with recurrent pneumothorax]. 1926 May 33

We reported the clinical features of 4 cases with pulmonary Langerhans' cell histiocytosis. All of them were men who had a history of smoking. They ranged in age from 23 to 46 years. Cases 1 and 3 did not stop smoking, while Cases 2 and 4 did stop smoking. All of the chest CTs revealed small nodules and cysts, and during the follow-up period of 8 to 13 years, the numbers of nodules decreased and the walls of the cysts became thin in all of the cases. Their pulmonary function tests revealed restrictive impairment in 3 cases and reduced diffusion capacity in 2 cases, but none showed obstructive impairment. FEV1% and DL(CO) deteriorated during the follow-up period. Case 1 died of respiratory failure after 8 years. Cases 2 and 4 were both alive with home oxygen therapy after 11 and 12 years, respectively. Case 3 was alive but complained of dyspnea on exertion after 13 years. In conclusion, all of the 4 cases showed deterioration of pulmonary function, and one of them died due to respiratory failure. The prognosis of patients with pulmonary Langerhans' cell histiocytosis appears to be poor.
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PMID:[Long-term follow up study of four cases of pulmonary Langerhans' cell histiocytosis]. 1976 8

A 31-year-old man presented with a dry cough and exertional dyspnea. The chest X-ray showed multiple nodular opacities throughout the entire lung field. Chest computed tomography (CT) revealed variable-sized nodules with a peribronchiolar or centrilobular distribution, some of which revealed thick-walled cavitary change. Based on the chest CT findings, it was initially assumed that metastatic lung nodules with hematogenous spread were present; therefore, we performed an open lung biopsy. On microscopic examination, several compact cellular interstitial infiltrates composed of Langerhans' cells, eosinophils, and lymphocytes were observed. Immunochemically, the Langerhans' cells showed strong cytoplasmic staining for S-100 protein. Based on these findings, the patient was diagnosed with Langerhans' cell histiocytosis of the lung. High-resolution CT of the chest is a useful, sensitive tool in the diagnosis of pulmonary Langerhans' cell histiocytosis (PLCH). A typical radiologic finding of PLCH is irregularly shaped cysts. The radiological finding in this case of nodular opacities throughout the lung fields only without cysts is rare in PLCH. We report a case of PLCH with atypical multiple nodules mimicking hematogenous metastatic lung nodules.
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PMID:A case of pulmonary langerhans' cell histiocytosis mimicking hematogenous pulmonary metastases. 1994 41

Case 1 was a 22-year-old younger sister of identical twins who had smoked 10 cigarettes per day for 4 years since she was 18. She was admitted to our hospital due to dyspnea on exertion, dry cough and bilateral diffuse reticulonodular infiltrates on chest computed tomography (CT). Histological findings of transbronchial lung biopsy demonstrated nodular lesions consisting of spindle-shaped mononuclear cells. Immunohistochemical studies revealed that the cytoplasm of these mononuclear cells showed positive reactions to CD1a. Based on these findings, she was given a diagnosis of pulmonary Langerhans cell histiocytosis (LCH). She reduced the number of cigarettes smoked to 1 cigarette per week. After 6 months, her respiratory symptoms and radiographic abnormalities had improved. Case 2 was the elder twin sister of case 1. She had smoked 5 cigarettes per day for 4 years since she was 18. She presented no respiratory symptoms. However, as the diagnosis in case 1 was LCH, a chest CT was taken which revealed 3 small nodular lesions. After six months, there were no significant changes on chest CT. At the time of writing, she is 23-years-old and continues to smoke, however, has no respiratory symptoms.
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PMID:[Pulmonary Langerhans cell histiocytosis in identical twins]. 2068 13

A 34-year-old woman developed polydipsia, polyuria, amenorrhea and loss of pubic hair in 2001, but did not seek medical advice. On September 7th, 2009, she was admitted to our hospital complaining of acute exacerbation of dyspnea on exertion. Chest computed tomography (CT) showed multiple cystic lesions, predominantly in bilateral lower lung fields. Non-segmental, diffuse ground-glass attenuated areas and thickened bronchovascular bundles were also seen in bilateral lung fields. Pathological findings of lung specimens from a surgical lung biopsy (right S6 and S8) 14 years previously showed infiltration of S100 protein-positive histiocytoid cells in the bronchiolar wall. As a result, pulmonary Langerhans cell histiocytosis (PLCH) was diagnosed. Moreover, panhypopituitarism due to LCH was identified on endocrine testing. Dyspnea on exertion, reduction of carbon-monoxide diffusing capacity (D(LCO)) and ground-glass attenuation areas on CT were improved by smoking cessation alone, and she was discharged. However, similar acute deterioration of PLCH recurred 4 months after first admission. Her dyspnea on exertion, reduction of D(LCO) and ground-glass attenuation areas on CT were improved again by 500 mg/day methylprednisolone pulse therapy for 3 days. This case was a unique combination of panhypopituitarism and the appearance and disappearance of ground-glass attenuation areas on CT, paralleling PLCH disease activity.
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PMID:[A case of pulmonary Langerhans cell histiocytosis with panhypopituitarism]. 2140 Sep 8

A 44-year-old Japanese woman was admitted to our hospital because of dry cough and dyspnea on exertion. She had never smoked. She had been passively exposed to smoking by her husband and co-workers from the age of 21 (1984) to 33 (1996). She had previously developed pneumothorax twice, in 1985. On admission, computed tomography (CT) of the chest showed reticulonodular opacities predominant in bilateral upper lung fields, and pulmonary function tests revealed a decrease in vital capacity. The differential diagnoses were sarcoidosis, idiopathic pulmonary fibrosis and pulmonary Langerhans cell histiocytosis (PLCH). Video-assisted thoracic surgery was performed to make a definitive diagnosis. A histological specimen revealed the presence of CD1a-positive Langerhans cells in bronchiolocentric nodular lesions, leading to a diagnosis of PLCH. She was given 0.5 mg/kg bodyweight/ day oral prednisolone. Her symptoms disappeared with steroid maintenance therapy, and her vital capacity on pulmonary function testing was prevented from further deterioration. Based on the pathogenesis of PLCH, this case suggested that not only active smoking, but also passive smoking, played an important role in the development of PLCH.
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PMID:[Pulmonary Langerhans cell histiocytosis in a non-smoking Japanese woman]. 2148 54

Pulmonary Langerhans cell histiocytosis is an uncommon diffuse cystic lung disease in adults. In rare cases, it can involve extrapulmonary organs and lead to endocrine abnormalities such as central diabetes insipidus. A 42-year-old man presented with polyphagia and polydipsia, as well as a dry cough and dyspnea on exertion. Magnetic resonance imaging of the hypothalamic-pituitary system failed to show the posterior pituitary, which is a typical finding in patients with central diabetes insipidus. This condition was confirmed by a water deprivation test, and the patient was also found to have type 2 diabetes mellitus. Computed tomographic scanning of the lungs revealed multiple, irregularly shaped cystic lesions and small nodules bilaterally, with sparing of the costophrenic angles. Lung biopsy through video-assisted thoracoscopic surgery revealed pulmonary Langerhans cell histiocytosis. On a follow-up visit, only 1 year after the patient had quit smoking, clinical and radiological improvement was significant. Here, we report an uncommon case of pulmonary Langerhans cell histiocytosis that simultaneously presented with diabetes insipidus and diabetes mellitus.
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PMID:Pulmonary Langerhans Cell Histiocytosis in an Adult Male Presenting with Central Diabetes Insipidus and Diabetes Mellitus: A Case Report. 2650 47