Department of Medicine, Nassau University Medical Center, an affiliate of North Shore Long Island Jewish (NSLIJ) Health Care Systems, 2201 Hempstead Turnpike, East Meadow, NY, 11554, USA.
Clin Rev Allergy Immunol. 2018 Jun;54(3):353-365. doi: 10.1007/s12016-015-8487-6.
Mast cell activation syndrome (MCAS) involves the skin, gastrointestinal, cardiovascular, respiratory, and neurologic systems, classified as primary, secondary, and idiopathic. Earlier criteria for MCAS diagnosis included episodic symptoms with mast cell mediators affecting two or more organ systems with urticaria, angioedema, flushing, nausea, vomiting, diarrhea, abdominal cramping, hypotensive syncope, tachycardia, wheezing, conjunctival injection, pruritus, nasal stuffiness, decrease in frequency, severity, or resolution of symptoms with anti-mediator therapy including H/H receptor antagonists, anti-leukotrienes, or mast cell stabilizers. Laboratory data includes an increased validated urinary or serum markers of MCAS, documentation of an increase of the marker above the patient's baseline value during symptomatic periods on more than two occasions, or baseline serum tryptase levels that are persistently above 15 ng/mL. Laboratory data also includes an increase of the tryptase level above baseline value on one occasion. Other assays are 24-h urine histamine metabolites, PGD or its metabolite, and 11-β-prostaglandin F alpha. A recent global classification is a response of clinical symptoms, a substantial transient increase in serum total tryptase or increase in other mast cell-derived mediators, histamine or PGD2 or urinary metabolites, and agents that attenuate production or mast cell mediator activities. "Spectrum of MCAS disorders" has been proposed, highlighting symptoms' diagnostic tests and treatments.
肥大细胞活化综合征（MCAS）涉及皮肤、胃肠道、心血管、呼吸和神经系统，分为原发性、继发性和特发性。MCAS 的早期诊断标准包括：以肥大细胞介质影响两个或两个以上器官系统为特征的间歇性症状，伴有荨麻疹、血管性水肿、潮红、恶心、呕吐、腹泻、腹痛、低血压晕厥、心动过速、喘息、结膜充血、瘙痒、鼻塞、症状频率、严重程度或缓解的减少，抗介质治疗包括 H1/H2 受体拮抗剂、抗白三烯或肥大细胞稳定剂。实验室数据包括增加了经过验证的 MCAS 尿或血清标志物，在两次以上症状期期间标记物超过患者基线值的增加，或基线血清类胰蛋白酶水平持续高于 15ng/mL。实验室数据还包括一次基线值以上的类胰蛋白酶水平升高。其他检测包括 24 小时尿组胺代谢物、PGD 或其代谢物和 11-β-前列腺素 Fα。最近的全球分类是对临床症状的反应，血清总类胰蛋白酶或其他肥大细胞衍生介质、组胺或 PGD2 或尿代谢物的显著短暂增加，以及减弱产生或肥大细胞介质活性的药物。“MCAS 疾病谱”已被提出，突出了症状的诊断测试和治疗。
