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木村病

Kimura disease

ORPHA:482疾病

定义 英文原文（暂无中文）

A rare systemic disease characterized by chronic inflammation of unknown etiology and occurring predominantly in males in the second to third decades of life, mainly in Southeast Asian countries. Patients present with painless soft tissue swelling most commonly involving the head and neck region, usually with salivary gland involvement (parotid and submandibular glands) and regional lymphadenopathy. Frequent laboratory findings are peripheral eosinophilia and raised serum IgE. The diagnosis is confirmed histopathologically by the presence of well-formed lymphoid follicles with eosinophilic infiltration and vascular proliferation in the biopsy.

别名

嗜酸性淋巴肉芽肿

基本事实

遗传方式
未知
发病年龄
成年期
患病率
<1 / 1 000 000

临床表型 5

极常见 99–80%3

  • 淋巴滤泡增生 HP:0002729
  • 循环IgE水平升高 HP:0003212
  • 嗜酸性粒细胞增多症 HP:0001880

常见 79–30%1

  • 淋巴结肿大 HP:0002716

偶见 29–5%1

  • 唾液腺形态异常 HP:0010286

外部标识与链接

OrphanetMONDO:0018830GARD:6835ICD-10 D21.9ICD-11 4B2YClinicalTrials.gov 检索

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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）