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

The authors report the case of a young man with dissociated anterior pituitary insufficiency affecting somatotropic and corticotropic functions, presenting as hypoglycaemia as a result of the deficiencies mentioned, primary hypothyroidism and, in addition, pulmonary fibrosis of uncertain aetiology, pulmonary biopsy not having been carried out. The aetiology of this double pulmonary and endocrine involvement is presumably the same. Sarcoidosis seems unlikely, whilst histiocytosis X with pulmonary, anterior pituitary and/or hypothalamic and thyroid involvement is much more probable.
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PMID:[Hypoglycemia caused by anterior pituitary insufficiency associated with pulmonary fibrosis. Apropos of a case]. 121 62

Pulmonary histiocytosis X is an uncommon but important cause of pulmonary fibrosis and honeycombing in young adults. This article reviews the pathologic, clinical, radiographic, and high-resolution computed tomographic (HRCT) features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. The main pathologic feature of pulmonary histiocytosis X is peribronchiolar inflammation, leading to fibrosis and cyst formation. Although the diagnosis of pulmonary histiocytosis X may be suspected on the basis of chest radiographic findings, the HRCT findings of predominantly upper lobe nodules and cysts are virtually pathognomonic of this disorder. As pulmonary histiocytosis X progresses, the nodules decrease in number, leaving multiple thin-walled cysts. HRCT can be useful in visually estimating the proportion of lung involved and is also valuable in distinguishing histiocytosis from other disorders that produce nodules or cysts. Chest HRCT helps confirm the pattern seen at radiography and is valuable in establishing the correct diagnosis.
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PMID:Imaging of pulmonary histiocytosis X. 160 42

The diagnostic value of chest radiography and high-resolution computed tomography (CT) in chronic diffuse interstitial lung disease (CDILD) was assessed in 140 consecutive patients with diffuse infiltration of the lung visible at radiography. Radiographs and CT scans were separately read by three independent observers without knowledge of clinical and pathologic data. The observers listed the three most likely diagnoses and recorded the degree of confidence they had in their choice on a 0%-100% probability scale. Findings at radiography and high-resolution CT were recorded by each observer and were used for a stepwise discriminant analysis between diagnoses. First-choice diagnoses of all three observers that were made with a high level of confidence (probability, greater than or equal to 75%) were more accurate with CT than with radiography (P less than .001). The superiority of high-resolution CT over radiography was most obvious for histiocytosis X and sarcoidosis; in cases of pulmonary fibrosis, CT was not significantly different from radiography. The interobserver agreement for the proposed diagnosis was significantly better with high-resolution CT (P less than .001). Twenty-one of 26 radiographic findings and 21 of 25 CT findings were discriminant. Stepwise discriminant analysis revealed the superiority of CT over radiography, since the ranking of all findings showed that the four most discriminant findings, and eight of the first 12 findings, were revealed with CT.
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PMID:Chronic diffuse interstitial lung disease: diagnostic value of chest radiography and high-resolution CT. 200 62

To evaluate the usefulness of anti-T6 monoclonal antibody cell analysis in the assessment of diffuse lung disease, 77 bronchoalveolar lavages (BAL) were performed on 70 subjects: 18 normal smokers, 14 normal nonsmokers, 30 patients with chronic interstitial lung diseases (15 sarcoidosis, 12 idiopathic or associated pulmonary fibrosis, 3 histiocytosis X) and 8 patients with diffuse lung neoplastic disorders. The percentage of T6-positive cells was significantly higher in normal smokers than in normal nonsmokers (p less than 0.05). Positive T6 cells were absent or less than 1% in normal subjects, in patients with interstitial lung diseases and in patients with diffuse lung cancer, except in a case of desquamative interstitial pneumonitis, who had 2% of reacting cells. In contrast, such cells were always 3% or higher in the 6 BAL performed in histiocytosis X patients (p less than 0.05).
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PMID:Bronchoalveolar lavage analysis with anti-T6 monoclonal antibody in the evaluation of diffuse lung diseases. 263 45

Thoracoscopy was performed under local anesthesia in 419 patients suffering from a diffuse lung disease. In 85% of the cases diagnosis was clarified by thoracoscopy. All other cases were confirmed by means of an open lung biopsy. The best results were obtained in sarcoidosis of the stages II and III, the sensitivity being 0.98. Tumour-conditioned diffuse lung diseases were clarified in 88% of the cases; proof of an interstitial pulmonary fibrosis or interstitial pneumonia was established in 85% of the patients. Results regarding histiocytosis X were poor: thoracoscopic-bioptic proof was successful in only 42% of the patients. In 419 examinations we only detected a severe complication (air embolism). Drainage times were on the average between 4 and 5 days. On the whole, the method was characterised in the field of diagnosis of diffuse lung diseases by a high degree of sensitivity and satisfactory specificity. Both in respect of the invasiveness of the examination and its sensitivity it occupies an intermediate position between peripheral bronchoscopically obtained biopsy and surgical open lung biopsy, representing a valuable extension of the diagnostic instrumentarium if the indication is carefully considered.
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PMID:[Thoracoscopy in diffuse lung diseases]. 271 51

Cutaneous histiocytosis may take two principal forms. It is either a benign proliferative process or a relentless, progressive process with a poor prognosis. In histiocytic medullary reticulosis, histiocytes demonstrate nuclear atypia and the outcome is uniformly fatal. Benign cephalic histiocytosis X causes lesions similar to those of histiocytosis X, but Langerhans' cells are absent. In congenital self-healing histiocytosis X, the Letterer-Siwe-like cutaneous infiltrate contains Langerhans' cells, but the lesions heal spontaneously without treatment. The nodular cutaneous lesions of juvenile xanthogranuloma appear in infancy and resolve without treatment; however, the higher percentage (10%) of associated ocular lesions may lead to glaucoma and blindness. In histiocytosis X, the cutaneous lesions show a marked proliferation of Langerhans' cells, with prognosis dependent on the patient's age and the extent of organ dysfunction. Patients who survive the acute form of the disease may develop diabetes insipidus, growth retardation, pulmonary fibrosis, and biliary cirrhosis. A subtle immunologic defect has been identified in patients with histiocytosis X, yet the pathogenesis of the disease is still speculative. Familial disease occurring in early infancy should be differentiated from complete or partial immunodeficiency syndromes. Guidelines for evaluating patients with cutaneous histiocytosis are reviewed.
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PMID:Cutaneous histiocytosis syndromes. 299 40

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

Thirty-five cases of histiocytosis X in the National Children's Hospital were clinicopathologically studied. Fourteen cases were categorized in diffuse histiocytosis X, Letterer-Siwe type (DHX), 19 cases in multifocal eosinophilic granuloma (MEG) and 2 cases in unifocal eosinophilic granuloma (UEG). Nine of 14 DHX died, of which 6 died of opportunistic infection due to hypoproteinemia and pancytopenia, and 3 died of pulmonary fibrosis probably due to histiocytic infiltration and resultant lymphedema. Infiltration of histiocytes in the bone marrow, thymus and lungs, in addition to the lymphoreticular organs, was conspicuous in autopsy cases of DHX. Skin biopsy was valuable for diagnosis and the immunostaining with anti-S100 antibody was a good marker to characterize infiltrating histiocytes. Prognostic factors and effects of treatments were also evaluated. Only one of 19 MEG died of opportunistic viral infection, but a longer duration for treatment was usually necessary compared to that for DHX. Pathogenesis of histiocytosis X was discussed in relation to T-zone histiocytes.
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PMID:A clinicopathological study of histiocytosis X. 633 90

This paper reports on a 23-year-old man suffering from pulmonary fibrosis caused by localised histiocytosis X. Although chest film examination shows diffuse pulmonary involvement the patient is asymptomatic. No other organ systems are involved. The different forms and prognosis of histiocytosis X are discussed.
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PMID:[Pulmonary fibrosis in histiocytosis X]. 660 47

Although effective treatments are available for many children with LCH, there are many others for whom no definitive therapy yet exists. These patients include those with 1) multisystem disease and associated organ dysfunction, 2) chronic, relapsing disease, 3) new onset pituitary involvement associated with diabetes insipidus and 4) long-term complications such as pulmonary fibrosis, liver fibrosis or CNS involvement. This introductory paper discusses these clinical problem areas and then reviews several new therapeutic approaches including novel chemotherapeutic agents, immunosuppressive strategies, bone marrow transplantation and gene therapy.
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PMID:Treatment options--commentary. 807 10


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