Department of Pediatrics, Division of Allergy and Immunology, Virginia Commonwealth University Health Systems, 1000 E Broad Street, Richmond, VA, 23219, USA.
Curr Allergy Asthma Rep. 2017 Oct 7;17(11):80. doi: 10.1007/s11882-017-0748-4.
In this review, we examine the current understanding of the pathogenesis, clinical presentations, diagnostic tools, and treatment options of pediatric mastocytosis as well as the natural history of the disease.
We discuss the emerging concept of mast cell activation syndrome. Mastocytosis in children presents most commonly as isolated cutaneous lesions and is a relatively rare occurrence with excellent prognosis and spontaneous regression often occurring by adolescence. Systemic mastocytosis with organ system involvement is a more serious condition and is likely to persist into adulthood.
在这篇综述中,我们探讨了儿童肥大细胞增多症的发病机制、临床表现、诊断工具和治疗选择以及疾病的自然史的现有认识。
我们讨论了肥大细胞激活综合征的新概念。儿童肥大细胞增多症最常见的表现为孤立性皮肤病变,且预后良好,常自发消退至青春期。伴有器官系统受累的系统性肥大细胞增多症是一种更严重的疾病,可能持续到成年。