Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P15088 (mast cell)
 14,925 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Langerhans cells and their pathologic counterparts can be identified in paraffin sections using immunohistochemical staining for S-100 protein. This procedure is useful in confirming a diagnosis of Langerhans cell histiocytosis (LCH). However, many other cell types are also positive for S-100 protein. Positive staining for CD1 (Leu 6) supports a diagnosis of LCH, but requires frozen tissue. A panel of antibodies would be desirable in confirming a diagnosis of LCH, particularly if these antibodies could be used on paraffin-embedded material. We studied the pattern of staining for commercially available monoclonal antibodies MT1, MT2, MB2, and LN1, which were originally marketed as lymphocyte markers, using paraffin-embedded tissue sections of cases of LCH. In all 20 cases pathologic Langerhans cells stained positively with MT1 only. Various other S-100 protein-positive lesions were also examined with MT1 and were consistently negative for MT1. Other cutaneous histiocytic and mast cell lesions were positive with MT1, but S-100 protein negative. Our results demonstrate that the monoclonal antibody MT1 serves as an additional marker for LCH and, together with S-100 protein, would make up a diagnostic panel of antibodies for LCH to be used on routine paraffin-embedded sections.
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PMID:Monoclonal antibody MT1: a marker for Langerhans cell histiocytosis. 219 Jan 98

Histiocytosis X and mastocytosis are proliferative processes that may have similar cutaneous manifestations. However, a positive Darier's sign (urtication on stroking of the lesion) is thought to reliably distinguish between these two diseases. We recently studied a 13-year-old girl with a 2-year history of extensive skin lesions and a positive Darier's sign. Routine histopathologic studies revealed a polymorphous cutaneous infiltrate composed of histiocytes, mast cells, eosinophils, and lymphoid cells. Electron microscopic studies demonstrated Langerhans granules in some of the histiocytes, and immunologic studies of frozen tissue showed that a significant subpopulation of the histiocytes marked as Langerhans cells. Giemsa staining of specimens from eight other cases of cutaneous histiocytosis X from our files revealed mast cells in all of the lesions, although none showed the abundance of mast cells present in the case with urtication. Our studies emphasize the often polymorphous nature of the cell population in cutaneous histiocytosis X and demonstrate that confusing clinical findings can result when the mast cell population in histiocytosis X produces urtication.
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PMID:Urticating histiocytosis: a mast cell-rich variant of histiocytosis X. 242 66

The spleen and lymph node are two of the most common organs involved in systemic mast cell disease (SMCD). However, SMCD infiltrates in the spleen and lymph node have a broad spectrum of morphological patterns which can make it difficult to recognize the diagnosis, especially when specimens are examined from patients in whom SMCD is not suspected. We reviewed the pathological features of 16 spleen and 23 lymph node specimens from 19 patients which represented all available material from a series of 58 Mayo Clinic patients with SMCD. The purpose of this study was to investigate the pathological manifestations of SMCD involvement in the spleen and lymph node and to address difficulties in differential diagnosis. All compartments of the spleen and lymph node were found to be affected by SMCD. SMCD lesions in the spleen were found in a paratrabecular (92%), parafollicular (69%), follicular (15%), and a diffuse red pulp (8%) distribution. In the lymph node, mast cell infiltrates affected the paracortex (88%), the parafollicular region (50%), the follicles (25%), the medullary cords (13%), and the sinuses (6%). Mast cells were frequently found in a perivascular location, and associated eosinophilia was common. Because of the broad spectrum of histological manifestations of SMCD in the spleen and lymph node, a wide range of differential diagnoses is discussed including follicular lymphoma, T-cell lymphoma, monocytoid B-cell hyperplasia and lymphoma, Kaposi's sarcoma, and Langerhans' cell granulomatosis.
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PMID:Pathology of the lymph node and spleen in systemic mast cell disease. 323 90

Fibrotic lung disorders are chronic inflammatory diseases in which inflammatory processes in the lower respiratory tract injure the lung and modulate the proliferation of mesenchymal cells that form the basis of the fibrotic scar. The pathogenesis of fibrosis in fibrotic lung disorders remains unclear; however, recent attention has focused on the potential role of the mast cell in the genesis of fibrosis. To determine whether mast cells are implicated in the pathogenesis of lung fibrosis, mast cells were compared with the degree of fibrosis in transbronchial lung biopsy specimens from 49 patients with fibrotic lung disorders (16 sarcoidosis, 15 farmer's lung disease, 9 cryptogenic fibrosing alveolitis, 6 bronchiolitis obliterans organizing pneumonia, 3 histiocytosis X). In lung tissue of patients with fibrotic lung disorders, there was an increased number of mast cells in respect to the control group (98.6 +/- 7.7 vs 27.8 +/- 5.1 mast cells per square millimeter, p < 0.01). Mast cell counts in lung biopsy specimens were significantly correlated with the degree of fibrosis (r = 0.87, p < 0.001); 80.8 percent of mast cells were found in the alveolar septa, 9.6 percent within alveoli, 1.9 percent among alveolar lining cells, and 5 percent along blood vessels. No mast cells were located within alveoli in controls. Our data suggest that mast cells participate in chronic inflammation and that their presence is related to interstitial fibrosis in a much broader spectrum of fibrotic lung disorders.
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PMID:Mast cells in fibrotic lung disorders. 813 13

This is a descriptive screening of 46 examples of childhood histiocytic lesions and some of their look-alikes using a monoclonal antibody, p55, to fascin. Fascin, an actin-bundling protein, identifies dendritic cells in the blood and in tissues. Our aim was to test the diagnostic utility of the antibody in various lesions at different sites and to see whether the staining patterns give insight into the cell types involved. Fascin intensely stained the cells of juvenile xanthogranulomas (JXG), Rosai-Dorfman lesions, and soft tissue dendrocytomas. Normal Langerhans' cells and the cells of Langerhans' cell histiocytosis were unreactive. Their lack of fascin staining may be relevant to fascin being maturation as well as lineage related. Epithelioid and palisading granulomas were unstained, though an example of Kikuchi lymphadenitis had large numbers of dendritic-type cells that stained strongly. A reticulohistiocytoma of the skin was also unstained and look-alike lesions, Spitz nevi, and mast cell lesions did not stain. Two of three large-cell lymphomas (both CD30+) also had fascin reactivity. Even though fascin is not specific to dendritic cells, staining other cell types as well (false positive), and not entirely sensitive, dendritic cells such as tissue Langerhans' cells are unstained (false negative), there seems to be a consistency of staining in childhood histiocytic lesions. This may be of diagnostic use when read in the context of the tissue differential diagnosis. Whether fascin can serve as a marker for the dendritic cell lineage, or at least for some phases of dendritic cell lifecycle, is not answered by this survey.
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PMID:Fascin and the differential diagnosis of childhood histiocytic lesions. 1046 81