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)

Over the last twelve years, only three cases of a dissiminated form of histiocytosis X, in infancy, have been treated at the Institute for Mother and Child Health Care in Novi Sad. The clinical picture exhibits a very characteristic, prolonged (from two to nine months) first stage of the illness. Its general symptoms are: fever, general decline of the infant, seborrheic dermatitis, coughing and recurrent purulent otitis. In the second stage the symptoms are even more pronounced but they depend on localization and the functional deterioration of the organs involved: the lungs, liver, pancreas, skin, bone marrow and lymph nodes. The article underlines the importance of hematological, cytological and histiopathological analyses in diagnosis, and it gives the results for the cases in question. Particular emphasis is placed on the importance of a cytological analysis of the skin scarificate. Two of the patients in question were treated with antibiotics and corticosteroids, while the third received antibiotics, corticosteroids and cytostatics, yet the outcome of all three cases was fatal. This is attributed, in part, to the late beginning of treatment and, in part, to the early age of the patients.
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PMID:[A disseminated form of histiocytosis X in infants]. 55 48

A case of pulmonary eosinophilic granuloma which was diagnosed by TBLB was presented. A 24-year-old male was admitted complaining of cough and fever of unknown etiology. He had a history of fever which had disappeared spontaneously 9 months previously. Laboratory examinations including blood gas analysis and pulmonary function tests showed no significant abnormalities. Chest roentgenogram showed faint linear opacities. Computed tomography revealed multiple small cysts and small nodular lesions mainly in the upper lung field. CT findings were strongly suggested pulmonary eosinophilic granuloma. TBLB was performed and 6 specimens were obtained, of which 4 showed granulomatous lesions consisting of histiocytosis X cell and inflammatory cells with infiltration of eosinophils. Furthermore, granulomatous lesions were positive for S-100 protein staining. The effectiveness of open lung biopsy has been emphasized in the diagnosis of pulmonary eosinophilic granuloma, however, TBLB is also useful for diagnosis, especially in the active or early stage of this disease.
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PMID:[A case of pulmonary eosinophilic granuloma diagnosed by TBLB]. 175 27

Histiocytosis X developed in five patients (one woman and four men) when aged between 15 and 44 years. The initial sign in four of them was eosinophilic granuloma of the bone, in one it was pulmonary involvement. In three patients the disease remained confined to bone, while in two it involved the lungs and central nervous system, respectively. Osteolysis regressed spontaneously in one of the men, while in the woman there has been no recurrence 8 years after resection of the focus. In another man an osteolytic focus in a rib was noted after a 9-year recurrence-free interval. The man with pulmonary and bone involvement received chemotherapy with vinblastine and prednisone: dyspnoea and cough disappeared, vital capacity improved and the interstitial lung changes regressed. The osteolytic foci were repeatedly irradiated in the man with bone and CNS involvement. This brought about considerable reduction in pain but no significant radiological changes of the foci. Two courses of chemotherapy were given over 12 years, once with vincristine and prednisone, afterwards with cyclophosphamide. This arrested the progression of the osteolytic foci, but each time they recurred when the drugs were stopped.
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PMID:[Histiocytosis X. Clinical aspects and course in 5 patients]. 193 52

The pulmonary primary localization of the histiocytosis X is rather rare. We describe here a case of histiocytosis X with a localization exclusively at pulmonary level in a 38 years old young man who is a heavy smoker. A dry cough, asthenia and an increasing dyspnoea were the first symptoms of the disease. The radiological picture showed a widespread pulmonary intersticepathy. The diagnosis has been obtained testing some fragments of the pulmonary parenchyma taken through a diagnostic thoracotomy with an electron microscope.
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PMID:[A case of primary pulmonary histiocytosis X]. 215 51

This is a case report of a 39 year old patient in whom histiocytosis X was discovered following a cough which had appeared several months before. The radiological investigations were distinctive immediately associating the alveolar lesions and the excavated macronodules. The clinical examination was normal but there were some laboratory features suggesting inflammation. The diagnosis was achieved following a surgical biopsy which on histological examination showed histiocytic granulomas and the immunology revealed cells which were positive for OK T6 monoclonal antibodies and S100 antiprotein antibodies. There was subsequently a spontaneous regression with disappearance of the nodular shadows only leaving a few cystic forms. This observation is distinctive and is characterised by the rare appearance of the initial radiology and also confirms the predisposing factor of smoking in the pathogenesis of histiocytosis X.
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PMID:[Histiocytosis X: excavated macronodular form. Initial alveolar phase with spontaneous regression. Role of smoking]. 227 Mar 49

A 22-year-old black male presented with progressive dyspnea, a nonproductive cough, and new skin lesions. He was severely hypoxic, and had a severe restrictive defect on pulmonary function testing. A 2-cm lytic defect was noted on skull radiographs. A lung biopsy demonstrated pulmonary fibrosis. A biopsy of a skin lesion was consistent with a diagnosis of multifocal eosinophilic granuloma, or disseminated histiocytosis X. The case presents several unusual features of this uncommon disorder.
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PMID:Multifocal eosinophilic granuloma. 326 19

A 50 year old male smoker was admitted with nonproductive cough, weight loss and bilateral infiltrates predominantly in the upper lung zones on chest X-ray. Before admission he had been treated for suspected tuberculosis without improvement. The definite diagnosis of pulmonary histiocytosis X was made by open lung biopsy. High resolution computed tomography revealed the typical "ring fixtures" distributed in the parenchyma. Diffusing capacity for carbon monoxide was markedly reduced, besides mild obstruction. After six months of observation, there was seen a radiological improvement with unchanged lung function parameters.
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PMID:Nonproductive cough, weight loss and bilateral pulmonary infiltrates. 808 17

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

A detailed retrospective analysis of 8 cases has been set up to obtain an update of the clinical, radiological, diagnostic and therapeutical aspects of pulmonary Langerhans' cell granulomatosis (PLCG), previously called Histiocytosis X. This disease represents 2.8% of interstitial lung diseases (ILD) in a registration by pneumologists in Flanders. Seventy five% of our patients were active smokers. Cough, dyspnoe and constitutional symptoms were the main presenting symptoms. There was a 37.5% frequency of pneumothorax during the whole disease evolution and all these patients had to be treated with chemical or surgical pleurodesis. Spirometric pattern was variable but CO-transferfactor (TLCO) was significantly impaired in all our patients. Radiologically nodules and/or cystic lesions were found with preserved volumes. Open lung biopsy led to the diagnosis in 6 of the 8 cases. Transbronchial biopsies (TBB) were found to be not sensitive. Systemic staging in our group showed 50% of the patients to have a second focus of organ involvement. Treatment with corticosteroids and possibly immunosuppressives was without clear effect. Since 25% of our patients ultimately became candidate for lung transplantation extrapulmonary staging is prerogative and the high frequency of pleurodesis procedures in the past medical history of our patients has important implications since bilateral pleurodesis is still a contraindication for lung transplantation in some but not all institutions.
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PMID:Pulmonary Langerhans' cell granulomatosis (histiocytosis X): clinical analysis of 8 cases. 986 60

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


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