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)

We report the results of a morphological analysis of 60 pulmonary biopsies gathered from a multi center study, organised by the clinico-pathological research group on Wegener's Disease under the auspices of the French Language Society of Thoracic Medicine. Forty of the sixty cases analysed were retained after indexing the histological aspects in order to specify their diagnostic value. Two groups of lesions were distinguished, which had different significance. Group A: These include the three major diagnostic criteria, which reinforce one another as they associate: 1) The polymorphoneutrophil microabscesses with limited central necrosis or an extended necrosis like the contours of a relief map. 2) An angiitis (arteries, veins, capillaries) with eccentric focal parietal crescent-shaped microabscesses. 3) Polymorphous granulomas with giant cells. Group B: In this group are the minor morphological observations (table II) of a lesser value and significance. 1) Acute or chronic lesions with alveolar haemorrhage, endogenous lipid pneumonia, xanthomatous granulomas, an organising pneumonia with an alveolitis. 2) Bronchial lesions: Bronchitis and necrotising bronchiolitis, which is more rarely follicular. 3) Sero-fibrinous or infiltrative neutrophil pleural lesions with focal microabscesses, elastolysis and elastophagia with giant cells in the elastic lamina. Thirteen cases presented with misleading lesions, which was a possible source of diagnostic error and led to a discussion of several associated disorders (Goodpasture's syndrome, and collagen disorder syndrome) or there may be systemic angiitis (Giant cell or lymphocytic) or also systemic or tissue eosinophilia (Churg-Strauss syndrome, bronchocentric granulomatosis) or necrotising bronchitis (atrophic polychondritis) or other forms of nodular interstitial fibrosis, such as histiocytosis X. We would like to stress the great polymorphic variation of the lesions and the difficulties which confront pathologists in the diagnosis of Wegener's Disease, above all when it is localised to the lung. There is value in finding at least one major diagnostic criteria which is associated with a minor criteria and with the help of the C.ANCA levels may lead to a narrow clinicopathological correlation and allows for a fairly precise approach to the diagnosis and identification of early or unusual lesions and thus to the early treatment of patients before irreversible renal failure appears.
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PMID:[Pulmonary lesions in Wegener's disease. Report of the French Anatomo-clinical Research Group. Study of 40 pulmonary biopsies]. 150 87

The accuracy of high resolution computed tomography (HRCT) in the diagnosis of pulmonary lesions arising in patients with leukemia, lymphoma, or other hematopoietic proliferative disorders was evaluated in postmortem lung specimens from 35 patients with one of these neoplasms. Lungs were obtained from autopsied patients and prepared in a manner that allowed for direct pathologic-radiologic correlation. Eighty-eight pulmonary lesions with one of five radiographic patterns were identified in these 35 lungs. The gross, histologic, and radiographic changes were examined and the radiologic and pathologic diagnoses for each lesion were compared. A diffuse alveolar pattern on HRCT was primarily the result of pneumonia or intraalveolar hemorrhage, while irregular focal densities were produced by foci of hemorrhage, infarcts, lymphoma, and fungal infections. HRCT of the lung from a patient with pulmonary histiocytosis X showed a wedge-shaped subpleural density in association with a "crab-like" interstitial pattern, which was histologically shown to be an infarct with interstitial enlargement by infiltration of histiocytosis X. Air bronchograms were seen in two settings. In patients with necrotizing pneumonia, air bronchograms were associated with bronchiectasis. In contrast, air bronchograms seen in a patient with a bronchocentric lymphoproliferative disorder showed a pattern of bronchial constriction. These results suggest that HRCT may play an important role in the evaluation of pulmonary lesions in patients with leukemia, lymphoma, or other hematopoietic malignancies.
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PMID:High resolution computed tomography of inflation-fixed lungs: pathologic-radiologic correlation of pulmonary lesions in patients with leukemia, lymphoma, or other hematopoietic proliferative disorders. 154 Aug 57

The interstitial lung diseases are comprised of a group of pulmonary disorders characterized clinically by diffuse infiltrates on the chest radiograph and histologically by distortion of the gas exchanging portion of the lung. The physiologic correlates are restriction of lung volumes and impaired oxygenation. The term "interstitial" when applied to these diseases is actually a misnomer because it implies that the inflammatory process is limited specifically to the area between the alveolar epithelial and capillary endothelial basement membranes. The diseases currently grouped as "interstitial" also frequently involve the alveolar epithelium, alveolar space, pulmonary microvasculature, and less commonly, the respiratory bronchioles, larger airways, and even the pleura. The enormous differential diagnosis of interstitial lung disease can be made manageable by understanding that pneumoconiosis, drug-induced disease, and hypersensitivity pneumonitis account for over 80% of the responsible entities and can usually be identified from the patient's history. The nine remaining diseases/disease categories include: sarcoidosis, idiopathic pulmonary fibrosis, bronchiolitis obliterans-organizing pneumonia, histiocytosis X, chronic eosinophilic pneumonia, collagen vascular disease-associated interstitial lung disease, granulomatous vasculitis (Wegener's granulomatosis, Churg-Strauss syndrome, lymphomatoid granulomatosis), Goodpasture's syndrome, and pulmonary alveolar proteinosis. The diagnosis of a specific interstitial lung disease can be made via various means including the patient's history, specific serologies, bronchoalveolar lavage, transbronchial biopsy, and biopsy of extrathoracic tissues or open lung biopsy. A directed diagnostic approach can be formulated based on an understanding of these techniques and a thorough knowledge of the clinical presentations and specific diagnostic criteria for each of the major diseases. This monograph will serve as a guide for the clinician to use in evaluating and treating patients with interstitial lung disease. We begin by reviewing the clinical presentation, diagnostic criteria, and management of specific interstitial lung diseases excluding pulmonary infection, neoplasm, and sarcoidosis. Pneumoconiosis and drug-induced syndromes are not discussed in detail, but the agents responsible and pertinent exposures are presented in tabular form in the discussion of the general diagnostic approach.
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PMID:Interstitial lung disease. 199 45

The diagnostic value of immunocytochemical tests was analysed for 19 cases of pulmonary histiocytosis X (PHX) and eight of other types of fibrosing pulmonary disease (sarcoidosis, 3; exogenous allergic alveolitis, 3; chronic pneumonia, 1; fibrosing alveolitis, 1). The cellular, proliferative-fibroblastic and fibrocystic stages in the course of pulmonary changes were differentiated. PHX cells reacted with anti-S 100 protein in all stages. In three cases for which unfixed tissue was available, all PHX cells reacted with antibody Leu-6, and 35% of these cells also reacted with the proliferation marker Ki-67. The few S-100 positive cells from the eight controls were limited to peribronchial tissue. Thus the antibodies Leu-6 and S-100 are useful aids in the diagnosis of PHX.
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PMID:[Immunocytochemical studies in the diagnosis of pulmonary histiocytosis X]. 278 67

Clinical, morphological, ultrastructural and immunological studies were performed in a case of congenital self-healing non-Langerhans cell histiocytosis. The patient showed several aspects that have not been published before: a large nodule in the vulvar region, vesiculobullous elements and pneumonia (asymptomatic). The relationship of the vesicles and pneumonia to the histiocytic disorder is not clear. Ultrastructurally, worm-like (comma-shaped) particles, dense bodies and Birbeck granules were not found. Histiocytes were Leu-6 negative, and S100 (partly), Leu M3 and HLA-DR positive. Positive reactions were also obtained with anti-lysozyme and non-specific esterase. Several aspects of this case and of others described previously are discussed.
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PMID:Congenital self-healing non-Langerhans cell histiocytosis. 305 80

This is the report of a female infant ten weeks of age, who was admitted to our hospital with hyperpyrexia, hemolytic anemia and disseminated intravascular coagulation. The further course of the disease was characterized by: continuing hemolysis resulting in severe normochromic, normocytic anemia, unrelenting disseminated intravascular coagulation, increasing hepato-splenomegaly with hyperbilirubinemia and ascites. No causative infectious organism could be identified. The infant died at the age of 14 weeks from respiratory insufficiency. Autopsy revealed massive hepato-splenomegaly, ascites and bilateral pneumonia. Histologic evaluation demonstrated lymphohistiocytic infiltrates of the periportal areas of the liver, the spleen and lymphnodes. Meninges were infiltered by macrophages with ingested erythrocytes. Differential diagnosis includes an infection with leptospira icterohemorrhagica (Weils disease) and erythrophagocytosis observed after various viral infections. Also histiocytosis X or malignant histiocytosis has to be taken into consideration. The most probable diagnosis in our patient is that of familiar hemophagocytic reticulosis although the familiarity in our patient was lacking. Intra vitam diagnosis can only be established by liver biopsy which could not be performed in our patient due to the severe coagulation disturbance.
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PMID:[Familial hemophagocytic lymphohistiocytosis (case report)]. 358 93

The number and scope of published articles dealing with computed tomography of interstitial lung disease are limited. We present seven cases in which computed tomography detected the presence or extent of interstitial lung disease better than conventional radiography: two patients with histiocytosis X, one with bronchopulmonary dysplasia, one with bleomycin lung toxicity, and three with radiation-induced lung injury. The computed tomography appearance of histiocytosis X and bronchopulmonary dysplasia have not been previously described. Transverse computed tomography images provide information regarding stage of activity and nature of interstitial lung processes not available with standard imaging techniques. We advocate the use of computed tomography in the initial investigation and follow-up of patients with histiocytosis X. Post-radiation pneumonitis and fibrosis can be detected earlier with computed tomography as well.
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PMID:Computed tomography in interstitial lung disease. 378 Feb 63

We reviewed the Tumor Registry for 1981 at the Children's Hospital of Philadelphia to identify all the children with newly diagnosed cancer who were seen initially in the emergency department (ED). Of the 220 new patients listed, 16 (7.3%) sought initial care in the ED (1 per 4,500 ED visits). Seven had leukemia, five had non-CNS solid tumors (2 lymphoreticular, 1 Wilms', 1 neuroblastoma, and 1 ovarian), and four had CNS tumors. Among the children with leukemia, pallor (6) and decreased activity (4) were the most common complaints. Duration of symptoms ranged from 4 days to 3 weeks. Physical examination showed pallor (5), splenomegaly (4), fever (3), hepatomegaly (3), lymphadenopathy (3), and ecchymoses or petechiae (2). The complete blood count and peripheral smears were all abnormal. The five patients with non-CNS solid tumors had symptoms related to the location of their neoplasms. The patients with Wilms' tumor, neuroblastoma, and ovarian dysgerminoma had abdominal masses; the patient with lymphoma had a large, painful inguinal node; and the patient with histiocytosis X had an infiltrative rash, gingivitis, and pneumonitis. Of the four children with CNS tumors, three had headache, and one had an incidentally detected scotoma following head trauma. All four eventually had abnormal neurologic exams and computer tomographic scans, but two were discharged initially with psychiatric diagnoses. We conclude that cancer, although rare in children, occurs with greater relative frequency in the referral hospital ED than that predicted by published cancer rates from the referring hospital's ED.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Detection of cancer in the pediatric emergency department. 384 22

The histopathologic and ultrastructural features of intraluminal organizing and fibrotic changes were studied in open lung biopsies and autopsy specimens from 373 patients with interstitial lung disorders, including hypersensitivity pneumonitis (n = 44), idiopathic pulmonary fibrosis (n = 92), collagen-vascular diseases (n = 20), chronic eosinophilic pneumonia (n = 10), pulmonary histiocytosis X (n-90), pulmonary sarcoidosis (n = 62), pneumoconioses (n = 25), Legionnaire's disease (n = 5), drug- and toxin-induced pneumonitis (n = 4), radiation-induced pneumonitis (n = 2), lymphangioleiomyomatosis (n = 11), and chronic organizing pneumonia of unknown cause (n = 8). Three patterns of intraluminal organization and fibrosis were recognized: 1) intraluminal buds, which partially filled the alveoli, alveolar ducts and/or distal bronchioles; 2) obliterative changes, in which loose connective tissue masses obliterated the lumens of alveoli, alveolar ducts or distal bronchioles, and 3) mural incorporation of previously intraluminal connective tissue masses, which fused with alveolar, alveolar ductal, or bronchiolar structures and frequently became reepithelialized. All three patterns had common morphologic features, suggesting that, regardless of their severity, they resulted from a common pathogenetic mechanism, ie, the migration of activated connective tissue cells, through defects in the epithelial lining and its basement membrane, from the interstitial into the intraluminal compartment. Intraluminal buds were observed most frequently in hypersensitivity pneumonitis, chronic eosinophilic pneumonia, and organizing pneumonia of unknown cause. Mural incorporation and, to a lesser extent, obliterative changes were observed in most interstitial disorders and were very prominent in idiopathic pulmonary fibrosis. Mural incorporation and obliterative changes play an important role in pulmonary remodeling, especially when several adjacent alveoli and/or other air spaces are involved. Under these circumstances, intraluminal organization can mediate the fusion of adjacent alveolar structures by intraluminal connective tissue.
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PMID:Intraluminal fibrosis in interstitial lung disorders. 395 68

Bronchoalveolar lavage is a simple technique, complementary to fiberoptic bronchoscopy. The material yielded comprises cells and supernatant, and both components may provide information about the distal lung structures. A few counter-indications should be respected. Normally, the cell differential comprises: 93 +/- 5% alveolar macrophages, 7 +/- 1% lymphocytes and about 1% neutrophils, eosinophils and basophils. Total cellularity and cell differentials are altered in many conditions, according to different patterns, which permits a classification. The increase in numbers may relate to macrophages (smokers, pneumoconiotic disorders...), to lymphocytes (sarcoidosis, hypersensitivity pneumonitis...) and to neutrophils and/or eosinophils (fibrotic disorders, chronic eosinophilic pneumonia, histiocytosis X...). In the last disease, the diagnosis may be established by detecting Langerhans cells. In other conditions, bronchoalveolar lavage mostly provides a trend to diagnosis. Usually well tolerated, bronchoalveolar lavage may usefully be repeated for monitoring patients with chronic interstitial lung disorders. It also gives an insight into the pathogenesis of many pulmonary pathological processes.
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PMID:[Exploratory bronchoalveolar lavage]. 623 22


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