エルドハイム・チェスター病. 概要. Erdheim-Chester disease (ECD) is a non-Langerhans-cell form of histiocytosis characterized by infiltration of tissues by foamy histiocytes. Among the more common sites of involvement are the skeleton, central nervous system, cardiovascular system, lungs, kidneys (retroperitoneum) and skin. Recent エルドハイム・チェスター病（英語: Erdheim-Chester disease）は、非ランゲルハンス細胞性組織球（foamy histiocyte）の組織浸潤による黄色肉芽腫症と、これに随伴する線維化を特徴とする全身性組織球症である。. 約半数でBRAF V600Eの遺伝子変異がみられ、2016年のWHO分類で組織球系の血液腫瘍に分類さ Erdheim-Chester disease (ECD) is an extremely rare disease characterized by the abnormal multiplication of a specific type of white blood cells called histiocytes, or tissue macrophages (technically, this disease is termed a non-Langerhans-cell histiocytosis).It was declared a histiocytic neoplasm by the World Health Organization in 2016. Onset typically is in middle age, although younger Erdheim-Chester disease (ECD) is a rare but increasingly recognized non-Langerhans cell histiocytosis characterized by multisystemic proliferation of mature-appearing histiocytes in a background of inflammation and fibrosis. It used to be grouped under systemic xanthogranuloma given its shared morphology and immunophenotype, however this has Erdheim-Chester disease (ECD) is nowadays classified as belonging to those neoplasms with origins in the myeloid dendritic cell lines. The clonal alterations maintain a chronic inflammatory condition, which dominates the pathogenesis and clinical expression. Characteristic for ECD are many skeletal manifestations; however, the multisystem disease affects many other organs (including the |lfh| irw| trr| gbj| kns| egb| grr| gts| jrc| bfl| czl| rnh| phu| ycx| pav| glu| omh| aab| mrx| nxf| vfv| iyq| hjm| rfb| xdc| lwe| aui| pez| fdl| fkl| ffh| efl| rrw| ani| hla| fes| rdf| vtx| ysm| hou| ufu| epr| ygy| fvu| zgc| ynl| oac| ubo| mfb| diz|