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Pulmonary Langerhans cell histiocytosis

ORPHA:687733疾病亚型暂无中文名

定义 英文原文(暂无中文)

A form of Langerhans cell histiocytosis characterized by interstitial changes in the lung tissue, manifesting as a variable combination of cellular inflammation, cystic, and fibrotic lesions. Patients can have symptoms like dyspnea, cough and fever but most of them are asymptomatic, and they may undergo spontaneous remission. It is predominantly observed in young smokers or ex-smokers.

别名

Single-system pulmonary Langerhans cell histiocytosis、Single-system pulmonary histiocytosis X、PLCH、Single-system Langerhans cell granulomatosis

基本事实

遗传方式
不适用

相关基因 1

基因名称关联类型
BRAFB-Raf proto-oncogene, serine/threonine kinaseDisease-causing somatic mutation(s) in

外部标识与链接

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本页数据来源

  • 疾病定义、同义词、基因、表型、流行病学:Orphanet(CC BY 4.0)
  • 中文病名:Orphanet 中文包,冻结于 2020-06-01;本条目在该版本中无中文名
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)