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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 expression of macrophage antigens KP1, Mac, lysozyme, and alpha-1-antichymotrypsin was investigated on routine paraffin sections from 17 cases of Langerhans' cell histiocytosis (LCH). All the major clinical forms were represented, including single lesions and monosystemic and multisystemic disease. In all the cases, a variable fraction (3-35%) of LCH cells was immunoreactive with KP1 and anti-Mac; the staining pattern was quite typical because the immunoreaction product was often confined to the perinuclear space and the Golgi area. LCH cells containing lysozyme and AACT were detected less frequently; however, in positive cases the percentage of LCH cells immunoreactive for lysozyme and AACT was in the same range as that of KP1-positive cells. On immunostained cytosmears (one case), about 10% of the CD1a-positive cell population was reactive for the macrophage antigens CD14 and PAM-1. No association was noted between the number of KP1-positive cells and the clinical form and/or anatomic site of the lesion. Phagocytic macrophages were significantly and diffusely immunoreactive with KP1 and anti-Mac and for AACT and lysozyme. Multinucleated giant cells with irregular nuclei were frequently observed; these cells were rarely S-100 positive, were consistently stained by KP1 and AACT, and were occasionally anti-Mac positive. The authors' findings suggest that antimacrophage monoclonals, in conjunction with S-100 protein, may represent a useful tool to establish the diagnosis of LCH in paraffin-embedded material.
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PMID:Expression of macrophage-associated antigens in tissues involved by Langerhans' cell histiocytosis (histiocytosis X). 278 88

Immunohistochemical examinations were performed using five kinds of histiocytic markers [S100 protein, lysozyme, non-specific cross reacting antigen with carcinoembryonic antigen (NCA), alpha 1-antichymotrypsin (alpha 1-ACT) and alpha 1-antitrypsin (alpha 1-AT)] in biopsied tissues from histiocytosis X, juvenile xanthogranuloma, xanthoma tuberosum, xanthoma disseminatum, reticulohistiocytic granuloma and multicentric reticulohistiocytoma, all of which have been classified as histiocytic proliferative disorders. Our results suggested that xanthomatous lesions of the skin to be composed of the histiocytic proliferation of two different cell lineages, i.e. S100+lyso-NCA- T-zone histiocytes and S100-lyso+NCA+ tissue macrophages. Only lesions of histiocytosis X were composed of the former cells. It is suggested that these markers will be useful in determining the delineation of the histiocytic system on the basis of functional heterogeneity.
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PMID:Immunohistochemical study on cutaneous histioproliferative lesions. 282 48

The morphological, ultrastructural and immunophenotypic properties of Histiocytosis-X (H-X) cells were investigated in a lymph node involved by Letterer-Siwe (L-S) disease. H-X cells were T6+ (CD1a), S-100+, T4+ (CD4) and HLA-DR+; in addition they were consistently T11+ (CD2) and were stained by antibodies directed against receptors for transferrin (T9), C3bi (OKM-1/CD11b), IgG-Fc (Leu-11/CD16) and Interleukin-2 (IL-2R/CD25). On immunostained cytosmears, T6+ cells were highly polymorphic and a prominent fraction (45%) showed immature morphology, characterized by lymphoid appearance. Cells expressing macrophage markers (ANAE, AACT, Leu-M3/CD14, PAM-1) were 10-fold fewer than T6+ cells and did not show a lymphoid morphology. At TEM level, H-X cells were characterized by poor content of LC granules and by the presence of myelin-like laminated bodies and of lysosome-like dense bodies. The immunophenotypic properties of H-X cells were compared to those of epidermal Langerhans cells (LCs) and of LCs present in lymph nodes of three cases of dermatophatic lymphadenitis. Epidermal LCs were T6+/HLA-DR+, and sometimes faintly T4+. Lymph node LCs were T6+, S-100+, T4+, HLA-DR+, and showed the same variety of surface receptors detected in H-X cells; furthermore, in a case with massive infiltration of the paracortex by T6+ cells, lymph node LCs were faintly T11+ and some of the T6+ cells had lymphoid aspect. Our findings suggest that the H-X cell population of L-S disease is not homogeneous, but is composed of discrete cell subsets with distinctive antigenic and morphological traits closely resembling those of cells of LC lineage at different maturational stages.
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PMID:Letterer-Siwe disease: immunohistochemical evidence for a proliferative disorder involving immature cells of Langerhans lineage. 313 61

The authors describe a 63-year-old woman who developed a histologically distinctive malignant cutaneous neoplasm composed of large pleomorphic cells with abundant cytoplasm and multilobate, often clefted nuclei that occasionally contained small nucleoli. This neoplastic cell population metastasized to a regional lymph node already involved by a B-cell derived chronic lymphocytic leukemia expressing surface IgMk, BA-1, and OKT1. The large metastatic tumor cells lacked surface immunoglobulin, B-lymphocyte associated antigen BA-1, T-lymphocyte associated antigens OKT1 and OKT3, and the monocyte/macrophage markers lysozyme and alpha 1-antichymotrypsin. These tumor cells expressed HLA-DR antigens, adenosine triphosphatase (ATPase), OKT6, and contained S-100 protein, i.e., they expressed the phenotype peculiar to epidermal Langerhans cells. The typical clinical and histologic features of Histiocytosis X were absent. Thus, this case appears to represent a distinctive cutaneous neoplasm composed entirely of malignant cells of dendritic cell origin which, by immunophenotypic and histochemical analysis, appear to be related to epidermal Langerhans cells.
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PMID:A distinctive cutaneous malignant neoplasm expressing the Langerhans cell phenotype. Synchronous occurrence with B-chronic lymphocytic leukemia. 388 25

The cellular nature of the proliferating histiocytes in 6 cases of histiocytosis X was studied immunohistochemically and ultrastructurally. Immunohistochemically, S-100 protein was detected both in the cytoplasm and the nuclei of histiocytosis X cells as well as Langerhans cells in normal oral epithelium. These cells were always negative for lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and immunoglobulins. S-100 protein was not detected in lysozyme-positive histiocytes and multinucleated giant cells often showed the signs of phagocytosis. Thus, S-100 protein appears to be a useful immunohistochemical marker for histiocytosis X cells. Ultrastructurally, Birbeck granules noticed in histiocytosis X cells were never seen in the phagocytic histiocytes with numerous lysosomes and phagosomes. These results emphasized the heterogeneous nature of the proliferating histiocytes involved in the lesions. Since histiocytosis X cells share characteristics, not only ultrastructurally but also immunohistochemically, with Langerhans cells, the hypothesis that histiocytosis X may be fundamentally an abnormal proliferation of Langerhans cells has been further supported.
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PMID:Immunohistochemical and ultrastructural analysis of the proliferating cells in histiocytosis X. 636 33

Histiocytosis X of the stomach of a 47-year-old Japanese woman, who underwent subtotal gastrectomy following a clinical diagnosis of scirrhous carcinoma, was studied by light and electron microscopy as well as by immunohistochemistry. The histiocytoid cells proliferated monotonously in the lamina propria mucosae of the atrophied mucosa covering the body and fornix. They were arranged in a sheet- or pavement stone-pattern and included some giant cells. The histiocytoid cells had a reniform to irregularly indented nucleus and conspicuous cytoplasm. Ultrastructurally, they were characterized by interdigitating cytoplasmic extensions and abundant tubulovesicular structures including Langerhans granules. S-100 protein, alpha 1-antitrypsin, and alpha 1-antichymotrypsin were immunohistochemically identified in the cytoplasm. Endoscopic biopsies of the extragastric digestive tract, a biopsy of the lymph node, and bone marrow aspiration excluded a systemic disorder. The case is regarded as benign localized histiocytosis X of the stomach, a previously undescribed gastric lesion.
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PMID:Benign histiocytosis X of stomach. Previously undescribed lesion. 660 21