高IgE综合征

• 作者: 何颖瑶 刘斌
• 来源期刊: 临床耳鼻咽喉头颈外科杂志
• 学科: 医学
• 关键词: 高IgE综合征 STAT3 DOCK8 TYK2
• 年，卷（期）: 2017,（11）
• 所属期刊栏目: 综述
• 页码范围: 892-896
• 页数: 5页
• 分类号: R593.3
• 语种: 中文
• DOI: 10.13201/j.issn.1001-1781.2017.11.019

摘要

A hyper-IgE syndrome is a rare immunodeficiesncy disease,often accompanied by high serum IgE.It often characterized by facial features,repeated skin infections,eczema and pulmonary infection,including autosomal dominant HIES(AD-HIES) and autosomal recessive HIES(AR-HIES).AR-HIES is caused by mutations in STAT3,which is presented as connective tissue,bone,vascular disease,and high brain white matter signal.ADHIES is mainly caused by mutations in DOCK8 and TYK2,which is presented as severe viral infection and poor prognosis.The treatment for Hyper-IgE syndromes is mainly to control infection,skin care and other symptomatic treatment,if necessary,should be done as early as possible hematopoietic stem cell transplantation.This article reviews the clinical manifestations,pathogenesis and treatment of high IgE syndrome.