系统性肥大细胞增生症

Systemic mastocytosis

肥大细胞增生症是骨髓增殖性肿瘤的一种类型,系统性肥大细胞增生症(SM)是肥大细胞在真皮组织以外的一个或多个系统内克隆性增生。因为常并发克隆性血液系统非肥大细胞疾病,在诊断中易被忽视,在2017年世界卫生组织(WHO)淋巴造血系统肿瘤分型中,SM增加了新的亚型。随着新的治疗药物,如米哚妥啉(PKC412)的研究,SM的治疗效果较前有所改善。

Mastocytosis is a heterogeneous disease, systemic mastocytosis is defined by compact multifocal mast cell infiltrates in bone marrow or extracutaneous tissues and other minor SM criteria, with or without skin involvement. The diagnosis of SM is difficult for complicated by hematologic nonmast cell lineage disease. In the updated WHO classification 2016, a new category is proposed. Tyrosine kinase inhibitors, multikinase inhibitor and antibody-drug conjugate mayhave potential efficacy in the treatment.