Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We have investigated the secretory function of cell suspensions from bone eosinophilic granulomas surgically collected in two patients with histiocytosis X. Unseparated cell preparations spontaneously produced interleukin 1 (IL-1) and prostaglandin E2 (PGE2). In order to ascertain that this secretion was due to the characteristic Langerhans cell-like histiocytosis X cells predominantly found in the bone lesions, we have purified T6+ cells by the use of a fluorescence-activated cell sorter. Such highly purified cell preparations were found to secrete IL-1 and PGE2 spontaneously in culture. Stimulation with endotoxins and treatment with interferon gamma (IFN gamma) revealed an intense IL-1 secretory function of histiocytosis X cells. Since both IL-1 and PGE2 are able to induce bone resorption in vitro, our findings are compatible with the hypothesis that histiocytosis X cells are responsible for the typical osteolytic lesion observed in histiocytosis X through the local secretion of these two mediators.
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PMID:Histiocytosis X. Purified (T6+) cells from bone granuloma produce interleukin 1 and prostaglandin E2 in culture. 241 61

In histiocytosis X (HX), which is regarded as a proliferative disease of Langerhans cells (LCs), the tumor cells share characteristic membrane antigens and ultrastructural features with normal LCs. To the present no markers have been described which distinguish HX cells from normal epidermal LCs. Here we report on the selective reactivity of HX cells with a monoclonal antibody against interferon gamma (IFNg). Our results show that HX cells share an epitope with human IFNg while normal LCs do not. It remains to be established whether the expression of IFNg is specific for HX cells or rather characterizes a certain activation state of LCs.
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PMID:Interferon gamma is a marker for histiocytosis X cells in the skin. 313 70

Granulomas are a local formation of specific cells including macrophages and lymphocytes revealing a chronic inflammatory reaction against infection, often due to intracellular agents. Granulomas can be composed of macrophages (foreign body reaction), epithelioid cells (immune granulomas of sarcoidosis, tuberculosis), or Langerhans' cells (histiocytosis X). In immune granulomas, the epithelioid cells derived from activated macrophages are found at the centre of the granuloma associated with CD4+ T-lymphocytes, while the periphery is mainly populated with CD8+ T-lymphocytes. Granulomas are not static formations, the rate of cell turnover is high. Different cytokines have been identified as mediators involved in the formation and maintenance of these granulomas: IL2, interferon gamma, TNF alpha, 1,25(OH)2D3, IL1. Major questions, especially concerning antigens involved in granulomatous reactions, remain to be answered for a better understanding of these events.
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PMID:[Physiology of granulomas]. 806 61