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Evans综合征

Evans syndrome

ORPHA:1959疾病

定义 英文原文(暂无中文)

A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.

别名

自身免疫性溶血性贫血伴自身免疫性血小板减少

基本事实

遗传方式
不适用
发病年龄
成年期、儿童期
患病率
1-9 / 1 000 000(Europe)

临床表型 13

必现 100%2

  • 自身免疫性溶血性贫血 HP:0001890
  • 自身免疫性血小板减少症 HP:0001973

极常见 99–80%2

  • 抗中性粒细胞抗体阳性的中性粒细胞减少症 HP:0001904
  • 瘀点 HP:0000967

常见 79–30%8

  • 瘀斑易感性 HP:0000978
  • 呼吸困难 HP:0002094
  • 鼻衄 HP:0000421
  • 疲乏 HP:0012378
  • 黄疸 HP:0000952
  • 昏睡 HP:0001254
  • 肌无力 HP:0001324
  • 苍白圈 HP:0000980

偶见 29–5%1

  • 晕厥 HP:0001279

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)