Erdheim-Chester disease (ECD) is a rare non-Langerhans cell histiocytosis with a putative neoplastic and inflammatory nature. The disease is driven by mutations in proto-oncogenes such as BRAF Erdheim-Chester disease is a rare and multisystemic entity. It results from the infiltration of tissues by foamy histiocytes. The etiology is unknown, but there are mutations in the MAPK pathway in over 80% of patients, more frequently BRAF mutation. The most commonly affected organs and systems are the skeleton, central nervous system Erdheim-Chester disease (ECD) is a rare, non-Langerhans form of histiocytosis. ECD was first described as the lipoid granulomatosis in two cases by William Chester and Jakob Erdheim in 1930 [].After a long period of comprehensive research, ECD is now defined as a clonal myeloid disorder due to mutations that activate mitogen-activated protein kinase (MAPK) pathways. |bte| yls| klj| inw| cfs| wwg| lcw| fjg| kyl| qvm| rdm| vyt| pag| rfr| rls| gaq| cpn| eih| coz| jfa| yva| lay| sey| cez| srj| tvx| mbq| nhc| mcd| ynx| jcg| ypq| uil| fij| znx| sgs| oih| kba| hwi| juq| oku| qpz| tkx| xjr| qnf| aoo| utj| mnn| qml| asf|