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成人和儿童皮肤肥大细胞增多症:新分类及预后因素

Cutaneous Mastocytosis in Adults and Children: New Classification and Prognostic Factors.

作者信息

Matito Almudena, Azaña José Manuel, Torrelo Antonio, Alvarez-Twose Iván

机构信息

Instituto de Estudios de Mastocitosis de Castilla La Mancha (CLMast), Hospital Virgen del Valle, Ctra. Cobisa s/n, Toledo 45071, Spain.

Department of Dermatology, Complejo Hospitalario Universitario de Albacete, Hospital General Universitario de Albacete, C/Hermanos Falcó nº 37, Albacete 02006, Spain.

出版信息

Immunol Allergy Clin North Am. 2018 Aug;38(3):351-363. doi: 10.1016/j.iac.2018.04.001. Epub 2018 May 17.

DOI:10.1016/j.iac.2018.04.001
PMID:30007456
Abstract

The skin is one of the most frequent tissues affected in patients with mastocytosis, but cutaneous lesions are highly heterogeneous in shape, size, color, number, localization, and distribution. The World Health Organization recognizes 3 subtypes of cutaneous mastocytosis (CM): maculopapular CM (MPCM), diffuse CM, and mastocytoma of skin. An international task force of experts in mastocytosis has recently proposed subdividing MPCM into monomorphic and polymorphic, which could predict the duration of the disease in children. More research is warranted to develop an improved classification of CM that ideally should incorporate robust factors with prognostic impact on disease behavior.

摘要

皮肤是肥大细胞增多症患者中最常受累的组织之一,但皮肤病变在形状、大小、颜色、数量、定位和分布上具有高度异质性。世界卫生组织认可皮肤肥大细胞增多症(CM)的3种亚型:斑丘疹型CM(MPCM)、弥漫型CM和皮肤肥大细胞瘤。肥大细胞增多症国际专家工作组最近提议将MPCM细分为单形性和多形性,这可以预测儿童疾病的持续时间。有必要开展更多研究以制定改进的CM分类,理想情况下应纳入对疾病行为具有预后影响的有力因素。

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