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经典型毛细胞白血病

Classic hairy cell leukemia

ORPHA:58017疾病

定义 英文原文(暂无中文)

A rare, slowly progressive, chronic leukemia characterized by presence of abnormal B-lymphocytes (medium sized with abundant irregular pale cytoplasm, hair-like cytoplasmic projections/ruffled cytoplasmic border, a round or bean-shaped nucleus and absent nucleoli) in the blood or bone marrow, spleen and peripheral blood pancytopenia, notable monocytopenia, and marked susceptibility to infection. The characteristic immunophenotype is CD11c+, CD25+, CD103+ and CD123+ with a BRAF mutation in most cases.

别名

白血病性网状内皮组织增殖

基本事实

遗传方式
未知
发病年龄
成年期
患病率
1-9 / 1 000 000(Europe)

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
  • 表型中文标签:HPO 简体中文翻译(CHPO 上游成果)