肥大细胞活化综合征

Mast cell activation syndrome

肥大细胞活化综合征(mast cell activation syndrome,MCAS)是由多种病因引起的一组谱性疾病,表现为由于肥大细胞介质释放而引起的发作性的多系统症状,可累及皮肤、胃肠道、心血管、呼吸系统和神经系统。严重的全身反应期间,基础血清类胰蛋白酶水平显著高于个体基线水平,也伴有其他肥大细胞相关介质水平升高,如尿前列腺素D2及其代谢物、尿组胺代谢物、尿中白三烯代谢物(白三烯E4)、肝素等升高。MCAS的诊断标准:(1)与肥大细胞活化相关的发作性症状影响两个或更多器官系统;(2)使用抗肥大细胞介质药物治疗可以降低症状发作的频率、严重程度或缓解症状;(3)在症状发生后4 h内,血清类胰蛋白酶水平≥120%基线水平+2 ng mL,和或其他肥大细胞相关生化指标升高。除避免诱因外,MCAS治疗多采用抗肥大细胞介质为主的综合治疗。

Mast cell activation syndrome(MCAS)is one group of spectrum disorders induced by diverse causes,which manifested by episodic multi-system symptoms mediated by release of mast cell mediators,resulted in affecting skin,gastrointestinal tract,cardiovascular,respiratory,and nervous system.The serum tryptase level is significantly increased during the course of severe systemic reactions compared to baseline,which is also accompanied by increase of the other mast cell-related mediators,such as prostaglandin D2 and its metabolites in urine,histamine metabolites in urine,leukotriene metabolites in urine(leukotriene E4),heparin,etc.Diagnostic criteria for MCAS include:(1)episodic symptoms associated with mast cell activation affect two or more organ systems;(2)treatment with anti-mast cell mediators can reduce the frequency and severity of the symptom onset,or relieve it;(3)within 4 hours of symptom onset,the serum tryptase level elevates by at least 20%compared to baseline plus 2 ng mL,and or other mast cell-related biochemical marker increases.In addition to avoiding triggers,treatment of MACS is a comprehensive treatment based on anti-mast cell mediators.