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

15 cases of histiocytosis X and 274 cases of histologically confirmed sarcoidosis were diagnosed during the investigation period from 1969 to 1975. Data of 12 adults with primary pulmonary histiocytosis X were evaluated in extenso. The necessary histological verification of diagnosis was only possible by open lung biopsy. Already in early stage small excavations were found by tomography in half of the cases. Eelvation of serum copper and of the index of leukocyte alkaline phosphatase was striking. In a single case antinuclear antibodies were proven. An intra patient comparison verifies corticosteroids suppressing the disease. On the occasion of a second lung biopsy in one case could be seen that after treatment no more histiocytosis-specific substrate was existing. Exacerbation and relapse during and after continuous long-term therapy were not observed. The features of histiocytosis X and sarcoidosis are set side by side in order to show differences and relations.
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PMID:[Special diagnostic and therapeutic aspects of pulmonary histiocytosis X in twelve cases from 1969 to 1975 (author's transl)]. 30 28

Closed trephine needle biopsy of the bone marrow has become an established procedure in the evaluation of many malignant and benign diseases in adults; however, its role in pediatric pathology has not yet been defined. In the period from February 1974 to April 1978 we have performed 164 such biopsies in 111 children under 15 years of age. A representative specimen has been obtained in over 80% of cases. This series included, in order of frequency, non-Hodgkin lymphomas, Hodgkin lymphomas, aplastic anemias, rhabdomyosarcomas, neuroblastomas, miscellaneous solid tumors, and single cases of histiocytosis X, malignant histiocytosis, sarcoidosis, malignant histiocytoma, and Castleman lymphoma of the hyaline-vascular type. Histology has been found superior to cytology in the detection of neuroblastoma invasion; the evaluation of the true cellularity in aplastic anemia, and the detection of granulomatous tissue in the only case of sarcoidosis. In other diseases histology and cytology gave similar information, except for the few cases of acute leukemia in partial relapse, which has been better defined in the aspirate smears than in the core specimen. Further evaluation of this technique in other patient series appears advisable.
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PMID:Bone marrow biopsy in children: a study of 111 patients. 44 Feb 5

Two cases of leptomeningo-cerebro(-spinal) sarcoidosis which were not clinically diagnosed in two women (22 and 33 years old) are described. The two women died because of this disease, 8 months, and 1 1/2 years, resp., after the first manifestation. In the first case there was a largely isolated local affection of the hypothalamus and neurohypophysis. In the second observation, beside an involvement of the bifurcate lymph nodes and of the lungs, it was only the central nervous system that showed massive diffuse changes partially due to a granulomatous angiitis of cerebral and visceral vessels. On the basis of pathohistology, the differential diagnosis to the histiocytosis X for case 1 and to the granulomatous giant cell angiitis for case 2 are discussed.
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PMID:[Cerebrospinal sarcoidosis (with reference to 2 personal cases)]. 90 97

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

Samples of lung biopsy tissue from nine patients with normal lungs, seven with pulmonary sarcoidosis, nine with diffuse interstitial pulmonary fibrosis, seven with histiocytosis X, and one with asbestosis were analyzed by morphometric techniques applied to electron micrographs, and the results were subjected to rank-sum analysis. Within the limits of the sampling method and the mathematical predictions of others, the general rejection of the constructed hypothesis in this study supports the conclusion that factors other than increase in the arithmetic of harmonic mean thickness of the blood-air barrier are critical to impaired gas transfer in the diseases studied.
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PMID:Fine structural morphometry of diffuse lung diseases with abnormal blood-air gas transfer. 124 96

High resolution computed tomography (TDM-HR) is now the technique of choice in the diagnosis and management of diffuse infiltrative lung disease (PID). After a brief review of the technique the authors describe the normal appearance; anatomical observations and the in vivo findings have shown that TDM-HR allow for the exploration of details of structure down to the second pulmonary lobule. Thus, through the alterations that are transmitted in the lobular area, and from its contents and its limits, PID has led to the elaboration of a new semeiology. The authors review the basic computed tomographic images and correlate these in each case with the histological evidence. The spatial distribution and the time sequence of the elementary images are the two other terms in the diagnostic equation of PID. The spatial distribution of several elementary images presents in TDM-HR a superior aetiological pointer to that which is furnished by thoracic radiographs; and the time sequence may furnish a useful indication as to the progress of the treated disease. Sarcoidosis, histiocytosis X, idiopathic interstitial fibrosis and lymphangitis carcinomatosis would serve as examples. Nevertheless, the authors point out that it would be dangerous during the period of evaluation to prematurely extend to all cases of PID conclusions which are only possible to make at present in a restricted number of disorders.
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PMID:[Diffuse infiltrative lung diseases: histological support of elementary lesions observed on tomodensitometry]. 150 84

In order to evaluate the diagnostic values of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBLB) in interstitial lung diseases, we made a retrospective study of a total of 43 patients all of whom underwent examinations for both conditions at the same time. In the BAL examination, fractional analyses of cell differential counts were applied to the first 30 ml lavage (FBAL-I, bronchial lavage) and to the following 50 ml aliquot of the second and third lavages (FBAL-II and -III, the latter supposedly alveolar lavage) lavages respectively. This technique revealed the unique characteristics of bronchial and alveolar inflammations in a separate data and this is assumed to be a precise reflection of the pathogenesis of interstitial lung diseases. The evaluation values (EV) were graded from 1 to 5 for both results according to the following diagnostic significance: EV1: not useful, EV2: normal or only slight changes from normal level, EV3: moderate changes although not diagnostic, EV4: quite compatible findings for diagnosis and EV5: definitely diagnostic. The 19 patients (43%) whose EV in TBLB were either 4 or 5 were diagnosed as having sarcoidosis (3), hypersensitivity pneumonitis (1), tuberculosis (2), pneumoconiosis (6), histiocytosis X (3), pulmonary infiltration with eosinophilia syndrome (PIE) (1), collagen vascular diseases (1), panbronchiolitis (1) or lymphagiomyomatosis (1). Thirteen of them had a BAL EV 4 which consisted of rather characteristic patterns of cell differential counts: thus their diagnoses were reconfirmed. In the other 4 patients with hypersensitivity pneumonitis, tuberculosis or PIE, the diagnoses were established by the findings of BAL EV 4 although the EV of TBLB were 3 or 2. These results indicate that BAL alone hardly establishes a diagnosis but it can support the TBLB findings and increase a diagnostic ability by 10% in total. Basophilic leukocytes, mast cells and Langerhans cells in BAL were rather non-specific, but the former two frequently appeared in allergic states and the latter two were often present in fibrotic lungs. Histiocytosis X is indicated if Langerhans cells are recovered from more than a small percentage of the total cell counts.
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PMID:[Diagnostic values of BAL and TBLB in patients with interstitial lung diseases]. 157 18

Fractional analysis of bronchoalveolar lavage (FABAL) fluid was performed in 6 control patients and 41 patients with various interstitial lung disease. The cell differential counts in the first 30 ml fraction of BAL (FBAL-I), which is considered to be the bronchial lavage, differed from those of the 50 ml second and third fraction (FBAL-III). Hypersensitivity pneumonitis, pulmonary tuberculosis, and sarcoidosis showed a high recovery of lymphocytes (52%); however, the former two disorders were occasionally, associated with neutrophil airway inflammation, whereas sarcoidosis was not. The percentage recovery of neutrophils in total FBAL was considerably high in patients with diffuse panbronchiolitis, and relatively high in those with collagen vascular disease, idiopathic pulmonary fibrosis, pneumoconiosis, and control smokers. However, these neutrophils were largely recovered from FBAL-I, suggesting the presence of airway inflammation. Thus, it is valuable to apply the FBAL method to determine the topographic distribution of inflammatory cells in the lungs. It was also found that the lymphocyte morphology in the lavage fluid was of value in establishing the diagnosis of hypersensitivity pneumonitis, and it is critical whether or not mast cells and basophils are present in BALF since they indicate the pathologic state of allergy or fibrosis. Although present in various fibrotic lung diseases in a limit number, langerhans cells are a diagnostic marker for histiocytosis X.
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PMID:[Airway and alveolar inflammation assessments with bronchoalveolar lavage in various interstitial lung disorders]. 163 46

The serum angiotensin-converting enzyme activity (ACE) was studied in 198 patients (116 had sarcoidosis and 82 pulmonary diseases with sarcoidosis-like X-ray picture). Rise of ACE activity in sarcoidosis patients was clearly associated with the process phase (93% in active sarcoidosis and 35.7% in nonactive). Rise of serum ACE activity was found in 46.3% of the patients having sarcoidosis-like diseases (in 41% with tuberculosis 56.3% with nonspecific inflammatory diseases and also in patients with fibrosing alveolitis, histiocytosis X and pneumoconiosis). Though the blood serum ACE activity has a high rate of the increase in sarcoidosis, this test should be considered only in the general complex of differential diagnosis signs. This can be explained by a sufficiently high probability of ACE activity rise in all diseases that most commonly require differentiation with sarcoidosis.
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PMID:[Serum angiotensin converting enzyme in the diagnosis of sarcoidosis and other lung diseases]. 166 54

Pulmonary histiocytosis X is characterized by an accumulation of CD-1-positive histiocytosis X cells in the lung, which also can be found in the bronchoalveolar lavage fluid (BALF). However, it has recently been demonstrated that CD-1-positive cells can also be detected in BALF of patients with other interstitial lung diseases and in healthy smokers. We therefore examined the frequency of CD-1-positive cells in a pool of patients with different pulmonary disorders, according to their smoking habits and diagnoses. We have studied the bronchoalveolar lavage in patients with pulmonary histiocytosis X (n = 6), sarcoidosis (n = 88), and in 97 patients with other miscellaneous lung disorders by using the immunoperoxidase method to detect CD-1-positive cells on glass slides. All patients with histologically proven histiocytosis X displayed more than 5% CD-1-positive cells, whereas patients with other pulmonary disorders showed no more than 3.6% CD-1-positive BAL cells. The dividing line of 5% CD-1-positive cells was not influenced by patients' smoking habits. The identification of CD-1-positive cells in BALF appears to be useful in diagnosing pulmonary histiocytosis X.
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PMID:Value of CD-1-positive cells in bronchoalveolar lavage fluid for the diagnosis of pulmonary histiocytosis X. 175


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