Article
Hematopoietic origin of Langerhans cell histiocytosis and Erdheim-Chester disease in adults.
Blood - 13 Jul 2017
Milne Paul, Bigley Venetia, Bacon Chris M, Néel Antoine, McGovern Naomi, Bomken Simon, Haniffa Muzlifah, Diamond Eli L, Durham Benjamin H, Visser Johannes, Hunt David, Gunawardena Harsha, Macheta Mac, McClain Kenneth L, Allen Carl, Abdel-Wahab Omar, Collin Matthew
Abstract excerpt
Langerhans cell histiocytosis (LCH) and Erdheim-Chester disease (ECD) are rare histiocytic disorders induced by somatic mutation of MAPK pathway genes. BRAFV600E mutation is the most common mutation in both conditions and also occurs in the hematopoietic neoplasm hairy cell leukemia (HCL). It is not known if adult LCH or ECD arises from hematopoietic stem cells (HSCs), nor which potential blood borne precursors...
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