Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P06126 (CD1a)
 2,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

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

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

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 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

A 53-year-old man, who was a heavy smoker, presented with recent severe cough. Radiography demonstrated a large pulmonary mass in the right upper lung. FDG PET/CT demonstrated heterogeneous high-grade activity in the pulmonary mass located in the right upper lung (standardized uptake value of 20), with central necrosis, bilateral upper mediastinal lymphadenopathy, right supraclavicular lymphadenopathy, direct left sternal manubrium invasion, and distal bilateral peripheral lung metastasis. Histology revealed significant malignant cytologic features and CD1a- and S-100-positive cells by immunohistochemistry staining, typical for Langerhans cell sarcoma.
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PMID:The characteristics of FDG PET/CT imaging in pulmonary Langerhans cell sarcoma. 2247 3

Isolated pulmonary involvement in Langerhans Cell Histiocytosis (LCH) is rare in childhood. The authors report a 2-y-old boy who presented with acute history of fever, cough and respiratory distress; later developed pneumothorax; whose CT thorax showed diffuse pulmonary cystic lucencies bilaterally. Lung biopsy confirmed pulmonary LCH with CD1a positivity. Recurrent spontaneous pneumothoraces are common in patients of pulmonary LCH; which necessitates rapid intervention. The authors share their experience of successfully using iodopovidone in pleurodesis for repeated pneumothorax episodes in this child with rare diagnosis of isolated pulmonary LCH.
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PMID:Iodopovidone pleurodesis for isolated pulmonary Langerhan's cell histiocytosis in a two year old child. 2403 94

Langerhans cell histiocytosis (LCH) is a rare clonal disorder of unknown etiology and characterized by the proliferation of dendritic cells. LCH most commonly involves the bone followed by the skin and the lymph nodes. Recently, only a few cases of LCH with predominant lymph node involvement have been diagnosed by fine-needle aspiration cytology (FNAC). A 2-year-old boy presented with generalized lymphadenopathy, fever, and cough. The patient had hepatosplenomegaly, anemia, and lytic lesions in the skull. FNAC from the largest submandibular lymph node showed features of LCH. The large cells of LCH showed positive immunostaining for S-100 protein on FNAC smears. Later, lymph node biopsy and immunohistochemistry against S-100 protein and CD1a confirmed the diagnosis of LCH. The patient was treated with chemotherapy and he is under regular follow-up. This case report highlights the importance of FNAC as a rapid and accurate investigation in the diagnosis of lymph node predominant LCH.
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PMID:Langerhans cell histiocytosis diagnosed by FNAC of lymph nodes. 2688 3