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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 morphological features of Langerhans cell histiocytosis (histiocytosis X) are characteristic but the diagnosis can on occasion be difficult. A case is presented that illustrates the diagnostic value of immunohistochemistry in the differential diagnosis of this condition. The cells of Langerhans cell histiocytosis were found to express CD1, CD4, CD11b and CD11c. They also reacted with EBM11, UCHM1, KB61 and HLA-DR. Occasional cells showed nuclear staining with Ki67, but no other lymphoid antigens were detected. Immunoreactivity of the cells of Langerhans cell histiocytosis with antibodies that recognize antigens present on macrophages provides further evidence for immunological similarities between these cell types.
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PMID:Langerhans cell histiocytosis: an unusual case illustrating the value of immunohistochemistry in diagnosis. 331 71

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

The antigenic properties of normal epidermal Langerhans cells and their neoplastic counterparts (histiocytosis X cells) were compared with those of other types of macrophages, by immunohistochemical staining of skin and lymph node biopsies with a panel of mono-clonal antibodies. The phenotype of Langerhans cells, histiocytosis X cells, dermal macrophages and interdigitating reticulum cells were similar (HLA-DR+, OKT 6+/-, Leu3+/-, C3-receptor-, Ig-complex-, R4/23-, MO2-), and differed markedly from those of follicular dendritic cells (HLA-DR-, OKT6-, Leu3-, C3 receptor+, Ig+, R4/23+, MO2-, and histiocytic reticulum cells (HLA-DR+/-, OKT6-, Leu3+/-, C3-receptor+, Ig-complex+, R4/23-, MO2+/-). This indicates that Langerhans cells are related to the interdigitating reticulum cells found in the T cell areas of lymphoid organs, and demonstrates that Langerhans cells do not express C3b, C3bi or C3d receptor molecules. Our data also emphasise that the Leu3 antigen is not restricted to cells of the T cell lineage.
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PMID:Normal and neoplastic Langerhans cells: phenotypic comparison with other types of macrophages. 387 94

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

Two elderly female patients are described with generalized histiocytosis X (Letterer-Siwe disease). In each case, a definitive diagnosis was not established until ultrastructural and immunoperoxidase investigations had been performed. The histopathological findings in skin biopsies from each patient were similar. Light microscopy demonstrated a bandlike epidermotrophic cellular infiltrate which included large atypical cells (histiocytosis X cells). Electron microscopy showed that these cells contained Birbeck granules. Monoclonal antibody studies demonstrated the presence of T6, T4 and HLA-DR surface antigens. Lysozyme and alpha-I-antitrypsin were absent from the cells. The associated cellular infiltrate included T4 and T8 positive lymphocytes. It is possible that more cases of generalized histiocytosis X in adults will be identified with the increasing use of specialized histopathological techniques and that the disease is more common than currently believed.
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PMID:Generalized histiocytosis X in the elderly: a light and electron microscope and monoclonal antibody study. 639 95

The monoclonal antibody KP-1 that recognizes the lysosome-associated glycoprotein CD68 was used together with antibodies to other antigens (actin, glial fibrillary acidic protein, keratin, neurofilaments, chromogranin, synaptophysin, S-100 protein, HMB-45, lysozyme, and HLA-DR) in a labeled streptavidin biotin immunoperoxidase method to phenotypically characterize 27 granular cell tumors, five schwannomas, five neurofibromas, two ganglioneuromas, three ganglioneuroblastomas, five carcinoid tumors, five malignant melanomas, and five examples of histiocytosis X. The neoplastic cells in all 27 of the granular cell tumors and four of the five schwannomas strongly stained for CD68, whereas none of the neurofibromas, ganglioneuromas, ganglioneuroblastomas, or carcinoid tumors contained CD68-positive tumor cells. These findings further strengthen previous observations, suggesting a histogenetic relationship between granular cell tumors and Schwann cells. KP-1 reactivity also was demonstrated in cells of histiocytosis X and malignant melanoma, complementing other studies that extend the tumor types positive in immunoperoxidase stains using this antibody.
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PMID:Immunohistochemical demonstration of the lysosome-associated glycoprotein CD68 (KP-1) in granular cell tumors and schwannomas. 854 22

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

An-11-day old girl was seen with brownish nodular lesions scattered over the body with emphasis on the face and scalp. Several lesions had started to involute. Tissue was studied by histopathology, immunohistopathology, routine electron microscopy, and immuno-electron microscopy using cryosubstitution and embedding in K4M. Immunohistopathology revealed that the cells of the dermal infiltrate were Langerhans cells. They expressed Leu 6 and HLA-DR. On routine electron microscopy no Birbeck granules were found in the dermal cells. Birbeck granules in epidermal Langerhans cells were deformed and often situated next to laminated dense bodies. The latter expressed Leu 6 and lysozyme on immuno-electron microscopy. It was concluded that congenital self-healing reticulohistiocytosis is a benign Langerhans cell disease in which Birbeck granules are transformed to laminated dense bodies and possibly degraded by lysosomal enzymes.
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PMID:Congenital self-healing reticulohistiocytosis--a benign Langerhans cell disease. 818 35

Langerhans cell histiocytosis (LCH), formerly histiocytosis X, is a rare disorder of unknown aetiology and pathogenesis which is characterized by clinical heterogeneity and an unpredictable course. LCH is considered to be a reactive, proliferative disease. The pathognomonic cell in the lesion has been shown to be identical or very similar to the Langerhans cell. Immunophenotyping studies have shown the cell to be CD1a (OKT-6), S-100 protein, HLA-DR, and CD4 positive; ultrastructurally, the presence of intracytoplasmic Birbeck granules is the hallmark of the entity. The lesions may be localized or generalized. Due to the lack of an accepted classification system for the stage of the disease and its rarity, very few comparative therapy studies have been carried out. Recently, the Histiocyte Society has suggested diagnostic criteria for LCH, and established a program of initial evaluation of the patient in order to start controlled treatment trials. Traditionally, chemotherapy has been preferred with a trend over time towards a more conservative approach. The rate of sequelae is high and connected to a chronic course of recurrent multiosseous disease. Case fatality is strongly associated to development of organ dysfunction seen in disseminated disease, which is especially seen at low age (< 2 years) of onset.
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PMID:[Langerhans-cell histiocytosis (histiocytosis X) in children]. 831 26

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


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