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)

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

Langerhans' cell histiocytosis (LCH) is an uncommon disease with variable manifestations. We report a case of LCH with the unusual initial presentations of chest pain and progressive heart failure in a 5-year-old boy. Chest radiography revealed a wide mediastinum with cardiomegaly. Electrocardiography showed first-degree atrioventricular block and an inverted T wave over V4-V6. Echocardiography, computed tomography, and magnetic resonance imaging of the chest all showed an infiltrating lesion that enveloped the entire heart, great vessels, and coronary arteries. Pathologic examination of the biopsy specimen revealed LCH. Chemotherapy, which included prednisolone, vincristine, methotrexate, and 6-mercaptopurine, had only a minimal effect on the tumor. After the addition of etoposide, the lesion decreased in size, and the symptoms and signs of heart failure and chest pain were ameliorated.
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PMID:Langerhans' cell histiocytosis presenting with a para-aortic lesion and heart failure. 1139

Pulmonary Langerhans' cell histiocytosis (LCH) represents an uncommon clinical disorder with unpredictable clinical presentation and outcome. Lung involvement may occur either in isolation or as part of a multiorgan disease. A 43-year-old woman was admitted to our hospital with acute left chest pain and shortness of breath. Spontaneous left pneumothorax was detected. All laboratory tests, including pulmonary function studies, were normal. Radiological findings posed high suspicion for LCH and lung biopsy confirmed this diagnosis. Further studies identified small cystic lesions in the scalp and liver. The diagnosis of multiple organ LCH involvement was made. Spontaneous pneumothorax might be the presenting clinical symptom of LCH. The present case emphasizes the capricious nature of LCH and the importance of an individualized therapeutic approach.
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PMID:A case of multiorgan Langerhans' cell histiocytosis presented with pneumothorax. 1558 54

We report a case with Langerhans cell histiocytosis appearing as an extra-pleural tumor. A 20-year-old man was transferred to our hospital because of right chest pain and fever. His chest X-ray showed an extra-pleural mass and chest CT scan showed a mass lesion with right 7th rib fracture. 67Gallium and bone scintigram showed uptake at the same site. We performed a CT-guided puncture biopsy. Pathological findings of the specimen showed diffuse proliferation of histiocytoid cells with some eosinophils. The histiocytes were positive for S-100 protein and CD1a on immunohistochemical stain. Langerhans cell histiocytosis was diagnosed. There was no other involvement of the disease except the rib. The tumor resolved only with smoking cessation and no recurrence was observed during the follow-up period. An association between smoking and progressions of the rib disease was suggested.
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PMID:[A case of Langerhans cell histiocytosis which resolved only on smoking cessation]. 1650 66

We report a case of multisystem Langerhans cell histiocytosis (LCH) with lung, bone and pituitary involvement. A 20-year-old man developed thirst, polydipsia and polyuria in 1983. He had right femur pain from 1988 and osseous LCH was diagnosed based on the operated specimen in 1989. From July 1990, he had right chest pain on coughing and dyspnea and was admitted in November 1990. LCH involving the lungs was diagnosed by CT images and diabetes insipidus was also detected. Steroid therapy was started from 1991, but he discontinued it in 1998. Though he stopped smoking, his clinical symptoms worsened and he experienced bilateral pneumothoraces in 2002 and since then he has been receiving home oxygen therapy. Pulmonary LCH is thought to have a good prognosis, but in recent studies, its survival rate appears low. We report a case of multisystem LCH with lung deterioration over about 15 years.
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PMID:[A case of multisystem Langerhans' cell histiocytosis with lung deterioration over 15 years]. 1684 15

We report a rare case of coexisting pulmonary adenocarcinoma and Langerhans' cell histiocytosis (LCH) in a 78-year-old woman who did not smoke. During follow-up of diabetes mellitus, she had complained of chest pain and was found to have a nodular lesion in S9 of the left lower lobe, which was resected surgically. No abnormal laboratory findings were obtained. Before surgical resection, needle biopsy specimens confirmed the existence of adenocarcinoma. The resected tumor in the left lower lobe was 3.0 x 1.8 x 3.0 cm, and histologically both acinar and bronchioloalveolar cell subtypes of adenocarcinoma were found in cancer foci. In addition to pulmonary adenocarcinoma, Langerhans' cell proliferation associated with marked eosinophil infiltration was incidentally found in a small nodule, approximately 3 x 2 mm in size in the subpleural region. The Langerhans' cells contained interdigitated nuclei, exhibiting rather clear nucleoplasm and cytoplasm; they were positive for S-100 protein, CD1a, and also CD4. Massive eosinophil infiltration was found around the focus of Langerhans' cell proliferation. This nodule appeared to be LCH. The adenocarcinoma and LCH were adjacent, and cancer cells were infiltrated only in the peripheral parts of LCH. The coexistence of adenocarcinoma and LCH appeared to be incidental. The association of adenocarcinoma and LCH is rare, and only several reports of it can be found in the English literature.
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PMID:A rare case of coexistence of pulmonary adenocarcinoma with Langerhans' cell histiocytosis. 1880 45

A 30-year-old man patient was admitted to our clinic with complaints including cough, dyspnea, and chest pain. He had been diagnosed with pulmonary Langerhans' cell histiocytosis (LCH) 9 years previously. HRCT of the thorax demonstrated the presence of diffuse emphysematous areas in both lungs with a honeycomb appearance, an increase in reticular density at the lower lobes, a mass lesion in the left hilus, and a mass lesion at the lower lobe of the left lung. Histological diagnosis of TBNA from the right lower paratracheal and subcarinal lymph nodes was adenocarcinoma. Chemotherapy was begun, but the patient died due to respiratory failure after two courses of chemotherapy. We present this case as an example of the rare development of bronchogenic carcinoma during the long-term course of LCH.
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PMID:Bronchogenic carcinoma developing during a long-term course of pulmonary Langerhans' cell histiocytosis. 1925 62

We report a rare case of Langerhans cell histiocytosis involving the sternum. The patient was a 12-year-old girl presenting with anterior chest pain and swelling. Radiographs and computed tomography showed an osteolytic lesion in the sternum. Technetium bone scintigraphy revealed increased uptakes in the sternum, the greater trochanter of the right femur, and the right distal tibia. Incisional biopsy for the sternum lesion was performed, and the histopathologic diagnosis was Langerhans cell histiocytosis. She was treated with chemotherapy and the symptoms disappeared.
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PMID:Langerhans cell histiocytosis of the sternum. 1939

Authors report a case of a 29-year old patient with pulmonary Langerhans' cell histiocytosis who presented with chest pain as a consequence of rib osteolytic process. We carried out a diagnostic work-up which included laboratory and radiographic analysis, lung function tests, bronchoscopy, cytologic and pathologic analysis. After reaching the diagnosis, corticosteroid therapy was introduced with long-term follow-up. In this report, we included a brief review of pulmonary Langerhans' cell histiocytosis.
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PMID:[Pulmonary Langerhans' cell histiocytosis--case report]. 1976 81

Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis most commonly characterized by symmetrical skeletal involvement and may present with pulmonary involvement leading to chronically progressive pulmonary symptoms. Characteristics on chest radiography include non-specific findings of diffuse interstitial and pleural thickening, micronodules, ground-glass opacities and parenchymal condensation as a result of infiltration by lipid-laden histiocytes. We present the case of a 50-year-old man with ECD presenting with acute pulmonary symptoms due to rupture of a large cystic lesion with resultant pneumothorax. He was brought by ambulance to our hospital, complaining of acute anterior chest pain and severe dyspnea. Chest radiography showed right-sided pneumothorax with a collapsed lung, a large, left-sided cystic lesion in the upper lung field and accentuated interstitial markings. Bullectomy and surgical biopsy were performed, demonstrating histologically histiocytic infiltrates that were strongly positive for CD68, but negative for S-100 protein and CD1a. Subsequent systemic examinations indicated widespread symmetrical skeletal involvement, leading to a definitive diagnosis of ECD.
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PMID:Erdheim-Chester disease presenting with pneumothorax. 2193 73


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