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

Proliferating cells in histiocytosis X (histiocytosis X cells) share many structural and immunophenotypic features with Langerhans cells, leading to the assumption that histiocytosis X represents a proliferative disorder of Langerhans cells. Because, depending on their state of activation and/or differentiation, Langerhans cells exhibit a varying immunophenotype, we investigated whether histiocytosis X cells display a similar phenotypic heterogeneity and, if so, whether the heterogenous biological behavior of histiocytosis X is reflected by differences in the immunophenotype of the proliferating cells. In 21 patients suffering from different clinical manifestations of histiocytosis X, proliferating cells uniformly expressed class I and II alloantigens, T200, CD1, CD4, and S100 protein. In 12 of 21 cases, histiocytosis X cells additionally exhibited immunocytochemically detectable amounts of C3b and C3bi receptors and certain monocyte/macrophage antigens (CDw14, Ki-M1, Ki-M6). This immunophenotypic heterogeneity of histiocytosis X cells could not be correlated with clinical course, prognosis, and final outcome of the disease in a given patient. The capacity of histiocytosis X cells to immunophenotypically mimic various states of Langerhans cell activation and/or differentiation, however, underscores the concept of histiocytosis X as a proliferative disorder of Langerhans cell origin.
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PMID:The phenotypic spectrum of histiocytosis X cells. 328 Jun 96

A 76-year-old man developed a generalized orange-red nodular eruption associated with constitutional symptoms. A biopsy specimen of a nodule revealed an extensive infiltration of histiocytes with relatively abundant cytoplasm and folded nuclei. Electron microscopy showed Langerhans' cell granules, which confirmed the diagnosis of histiocytosis X. Results of immunohistochemical studies revealed a pattern of antigen expression usually found in histiocytosis X, including Ia, T6, and S100. Analysis of the DNA content of the cells with flow cytometry revealed an aneuploid peak. The patient responded partially to topical mechlorethamine hydrochloride therapy.
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PMID:Histiocytosis X. Flow cytometric DNA-content and immunohistochemical and ultrastructural analysis. 351 12

Immunoperoxidase staining for S100 protein and HLA-DR antigen was used to identify histiocytosis X (HX) cells in 23 cases of pulmonary histiocytosis X (PHX), three cases of idiopathic pulmonary fibrosis, and one case of hypersensitivity pneumonitis. S100 protein was present in HX cells in 22 of the PHX cases; HLA-DR antigen was present in HX cells from 16 cases. Varying numbers of peribronchiolar and interstitial cells were positive for either S100 or HLA-DR in two of the three cases of idiopathic pulmonary fibrosis, and in the case of hypersensitivity pneumonitis. Immunoperoxidase staining for chromogranin showed isolated neuroendocrine cells within the mucosa and wall or airways, sites in which HX cells were occasionally found. As other types of dendritic cells, as well as some neuroendocrine cells, may contain S100 protein, positive staining for S100 is not specific for HX cells.
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PMID:Pulmonary histiocytosis X. Immunoperoxidase staining for HLA-DR antigen and S100 protein. 353 3

We studied a unique case of a localized non-Hodgkin's lymphoma of the pleomorphic large-cell type arising in the small intestine to determine its phenotype. Immunohistochemical staining for S100 protein, lysozyme, alpha 1-antitrypsin, and Leu-M1 was performed. Many lymphoma cells were positive for S100 protein and were negative with the other antibodies. These findings indicate a probable dendritic cell origin for this lymphoma similar to that seen in histiocytosis X and some cases of malignant histiocytosis, but apparently quite distinct from the S100 protein-negative, lysozyme-positive, alpha 1-antitrypsin-positive cells seen within the mononuclear phagocytic system.
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PMID:Dendritic cell phenotype in localized malignant histiocytosis of the small intestine. 389 79

Normal tissues and various tumors were examined for S100 protein, using anti-S100 protein antiserum, in an immunoperoxidase reaction. Among normal tissues, in addition to the previously reported presence of S100 protein in some neurons, glial, and Schwann cells of the nervous system, melanocytes and Langerhans cells of the skin, interdigitating reticulum cells of lymph nodes, and chondrocytes, we demonstrated it in myoepithelial cells and ducts of sweat glands, salivary glands, and the breast, serous glands of the lung, fetal neuroblasts, and sustentacular cells of the adrenal medulla. Among neoplasms, S100 protein previously has been reported in neurogenic tumors, melanomas, and neuroblastomas; we have demonstrated it in mixed sweat gland tumors, histiocytosis X, pleomorphic adenomas of the salivary gland, medullary carcinomas of the breast, bronchioloalveolar carcinomas of the lung, sustentacular cells of pheochromocytomas, teratomas of the ovary, and tumors of cartilage (enchondromas, osteochondromas, and chondrosarcomas). With S100 protein producing tumors, a normal progenitor cell was identified, indicating that demonstration of S100 protein in tumors confirmed their origin.
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PMID:Role of antibody to S100 protein in diagnostic pathology. 629 96

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

The distribution of S100 protein in normal skin and various tumors involving skin was assessed using rabbit antibody to S100 protein in an immunoperoxidase reaction. In normal skin, S100 protein was detected in the epidermis (melanocytes and Langerhans' cells), dermis (Schwann cells, Pacinian and Meissner's corpuscles, and interdigitating reticulum cells), cells of the sweat gland apparatus, and in chondrocytes. In tumors involving skin, S100 protein was present in nevi, malignant melanomas, histiocytosis X, mixed sweat gland tumors, neural tumors, chondromas, and chondrosarcomas. Detection of S100 protein by immunostaining was useful in understanding the histogenesis of various skin tumors and in assessing the diagnosis and prognosis of a variety of skin lesions encountered in surgical pathology.
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PMID:The value of immunohistochemical studies using antibody to S100 protein in dermatopathology. 637 34

A 62-year-old female with histiocytosis X presented with a vulvar ulcer. Multiple osteolytic lesions were later detected. Histological examination of the ulcerated skin showed diffuse proliferation of histiocytic cells with folded nuclei and pale eosinophilic cytoplasm. Immunohistochemistry revealed S100 protein and vimentin as well as CD1a, CD4, and HLA-DR antigens in the proliferating cells. Electron microscopy demonstrated Birbeck granules in the cytoplasm of the cells. The patient was successfully treated by complete surgical excision of the ulcer followed by radiotherapy for recurrent vulvar erythema.
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PMID:An adult case of histiocytosis X with a vulvar ulcer and multiple bone lesions. 805 99

A case of Erdheim-Chester disease which affected the epiphysis and showed evidence of systemic disease is presented. Clinical and histopathological similarities with other forms of disseminated Langerhans' cell histiocytosis are noted, particularly reaction of infiltrating histiocytes for S100 and HLA-DR.
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PMID:Erdheim-Chester disease with epiphyseal and systemic disease. 832 Mar 35

A solitary massive tumor developed in the left eye of a 61-year-old female who had a history of insulin-independent diabetes mellitus for 20 years and whose left eye had become blind due to repeated anterior uveitis in the recent several years. The eyeball was enucleated and used for further examinations. Intraocular tissues were widely infiltrated by histiocytes, though no extraocular invasion or further metastasis was found. Histological examination including alpha 1-anti-chymotrypsin and S100 staining revealed the features characteristic of histiocytosis X. This is probably a case of intraocular histiocytosis X with no evidence of systemic symptoms.
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PMID:[A case of intraocular histiocytosis X]. 833 72


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