Langerhans cell histiocytosis is a type of cancer that can damage tissue or cause lesions to form in one or more places in the body. Langerhans cell histiocytosis (LCH) is an inflammatory myeloid neoplasia caused by alterations (mutations) of several genes in the MAPKinase pathway. Dendritic cells are a form of histiocyte, or white blood cell. Langerhans cell histiocytosis (LCH) is a group of rare disorders that overproduce and accumulate certain types of white blood cells (histiocytes) in organs and tissues in the body. The medical condition known as Langerhans Cell Histiocytosis is a rare disorder that may cause damage to other organs of the body. Operative case of Langerhans'cell histiocytosis of the skull with dural invasion. The disease mostly affects the axial skeleton, namely skull, jaw bone, spine, pelvis, ribs, and long bones. [1] The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and organ dysfunction of the pituitary, lung, liver, and bone marrow. They can be found in the skin, lungs, stomach, bone, eyes and intestines. [2] Causes and pathogenesis remain unclear. An immunohystochemical study of ki-67 expression of eosinophilic granuloma: case report and review of the literature. Lesions in the long bones are primarily located in the diaphysis. Langerhans cell histiocytosis (LCH), previously called histiocytosis X, refers to a spectrum of disease characterized by idiopathic proliferation of histiocytes producing focal or systemic manifestations. BACKGROUND 1.1. These cells play a role in the body’s immune system. Langerhans cell histiocytosis is a disease of myeloid dendritic cells, lymphocytes, and macrophages mixed with eosinophils and neutrophils. Clinical spectrum of central nervous system involvement in Langerhans cell histiocytosis. It frequently involves the soft tissues adjacent to the bone. People with LCH produce too many Langerhans cells or histiocytes, a form of white blood cell found in healthy people that is supposed to protect the body from infection. Carangelo B(1), Peri G, Tacchini D, Mariottini A, Palma L. 1. Histiocytes are part of the body’s immune system. EG is the most common form of Langerhans cell histiocytosis. Langerhans cell histiocytosis (LCH) is a rare cancer that begins in LCH cells.LCH cells are a type of dendritic cell which fights infection.Sometimes there are mutations (changes) in LCH cells as they form. However, recent studies suggest abnormal immune regulation as an important factor. The symptoms experienced by patients depend on the organs that are affected [1, 2].. Langerhans cell histiocytosis (LCH) is a group of idiopathic disorders characterized by the presence of cells with characteristics similar to bone marrow–derived Langerhans cells juxtaposed against a backdrop of hematopoietic cells, including T-cells, macrophages, and eosinophils. Langerhans cell histiocytosis (LCH) is a disorder that primarily affects children, but is also found in adults of all ages. While the cause of LCH is unknown, LCH can frequently behave like cancer and so is treated by cancer specialists. Several important studies published from 2010 to 2016 have immensely clarified the biology of LCH. These include mutations of the BRAF, MAP2K1, RAS … In normal numbers, they help fight infection. Langerhans cell histiocytosis (LCH) is a neoplasm of pathologic dendritic cells (DC) expressing CD1a and CD207 (Langerin) surface antigens, which some consider a reactive immune disorder and others a neoplastic disorder. 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