肥大细胞活化综合征的最新诊疗进展  

Recent progress in diagnosis and treatment of mast cell activation syndrome

作　　者：隆海燕[1] 韦江红(指导)[1] LONG Haiyan;WEI Jianghong(Department of Respiratory and Critical Care Medicine,Affiliated Hospital of Guilin Medical College,Guilin 541000,China)

机构地区：[1]桂林医学院附属医院呼吸与危重症医学科,桂林541000

出　　处：《中国免疫学杂志》2023年第8期1781-1783,1788,共4页Chinese Journal of Immunology

基　　金：国家自然科学基金(81960007)资助。

摘　　要：肥大细胞活化综合征(MCAS)是临床罕见病,是由肥大细胞分泌的介质导致的自发性系统性过敏反应,同时影响至少两个器官系统的临床综合征。由于MCAS可累及多个系统,其症状不典型,易漏诊及误诊,而MCAS重症患者可危及生命,给临床诊断治疗带来巨大挑战。本文就MCAS诊断、治疗的最新进展及其与遗传性胰蛋白酶血症(HAT)的关系以及COVID-19流行下MCAS患者的治疗管理进行综述,以期提高临床对MCAS的认识。Mast cell activation syndromes(MCAS)is a clinical disorder characterized by spontaneous systemic anaphylaxis caused by mediators secreted by mast cells and affecting at least two organ systems simultaneously.MCAS can involve multiple systems,its symptoms are atypical,easy to miss diagnosis and misdiagnosis,and severe MCAS patients can endanger their lives,bringing great challenges to clinical diagnosis and treatment.In this review,the latest progress in diagnosis and treatment of MCAS,its relationship with hereditary trypsinemia(HAT),and management of MCAS patients under COVID-19 epidemic are reviewed,with a view to improving understanding of MCAS.

关 键 词：肥大细胞活化综合征 遗传性胰蛋白酶血症 COVID-19 

分 类 号：R593[医药卫生—内科学]