Langerhans cell histiocytosis (LCH) is characterized by an accumulation of dendritic Langerhans cells in granulomatous lesions in various organs. The etiology of LCH remains enigmatic. Fas/APO-1/CD95 belongs to the "death receptor" family of apoptosis regulators and has been implicated in the downregulation of immune responses.
View Article and Find Full Text PDFPurpose: Langerhans cell histiocytosis (LCH) is a disease with a variable clinical manifestation, being localised (SS) or disseminated (MS). The etiology and pathogenesis of LCH is unknown. It is a proliferative disorder of monoclonal origin, but not necessarily neoplastic.
View Article and Find Full Text PDFJ Pediatr Hematol Oncol
December 2002
Purpose: Langerhans cell histiocytosis (LCH) is a rare pediatric and adult disease causing skin rashes, osteolytic bone lesions, tumorous growth in various organs, and in some patients, organ dysfunction. The cause of the disease is obscure, and it is not yet understood why some patients develop single-system lesions only without relapse, whereas others develop fatal multiorgan disease. The expression of p53 tumor suppressor gene product detected immunohistochemically can be used as a guideline to alterations in DNA repair control and apoptosis.
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