Division of Rheumatology, Allergy, and Immunology, Brigham and Women's Hospital Mastocytosis Center, Boston, Mass.
Mayo Clinic Program for Mast Cell and Eosinophil Disorders, Mayo Clinic, Rochester, Minn.
J Allergy Clin Immunol Pract. 2019 Apr;7(4):1097-1106. doi: 10.1016/j.jaip.2019.02.002.
Patients with clonal mast cell activation syndromes (MCAS) including cutaneous and systemic mastocytosis (SM) may present with symptoms of mast cell activation, but in addition can have organ damage from the local effects of tissue infiltration by clonal mast cells. Patients with nonclonal MCAS may have chronic or episodic mast cell activation symptoms with an increase in serum tryptase and/or urinary metabolites of histamine, prostaglandin D2, and leukotrienes. Symptoms of MCAS and SM can be managed by blockade of mediator receptors (H1 and H2 antihistamines, leukotriene receptor blockade), inhibition of mediator synthesis (aspirin, zileuton), mediator release (sodium cromolyn), anti-IgE therapy, or a combination of these approaches. Acute episodes of mast cell activation require epinephrine, and prolonged episodes may be addressed with corticosteroids. Patients with clonal mast cell syndromes may need a reduction in the number of mast cells to prevent severe symptoms including anaphylaxis and/or progression to aggressive diseases.
患有克隆性肥大细胞激活综合征（MCAS）的患者，包括皮肤和系统性肥大细胞增多症（SM），可能会出现肥大细胞激活的症状，但除此之外，还会因克隆性肥大细胞对组织的浸润而导致器官损伤。患有非克隆性 MCAS 的患者可能会出现慢性或阵发性肥大细胞激活症状，血清类胰蛋白酶和/或组胺、前列腺素 D2 和白三烯的尿代谢物增加。MCAS 和 SM 的症状可以通过阻断介质受体（H1 和 H2 抗组胺药、白三烯受体阻滞剂）、抑制介质合成（阿司匹林、齐留通）、介质释放（色甘酸钠）、抗 IgE 治疗或这些方法的组合来治疗。肥大细胞激活的急性发作需要肾上腺素，而长时间的发作可能需要用皮质类固醇治疗。患有克隆性肥大细胞综合征的患者可能需要减少肥大细胞的数量，以防止出现严重的症状，包括过敏反应和/或进展为侵袭性疾病。
