系统性肥大细胞增多症

Systemic mastocytosis.

作者信息

Akin Cem, Metcalfe Dean D

机构信息

Laboratory of Allergic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA.

出版信息

Annu Rev Med. 2004;55:419-32. doi: 10.1146/annurev.med.55.091902.103822.

DOI:10.1146/annurev.med.55.091902.103822

PMID:14746529

Abstract

Systemic mastocytosis is a clonal disorder of the mast cell and its progenitor. The symptoms of systemic mastocytosis are due to the pathologic accumulation and activation of mast cells in various tissues such as bone marrow, skin, gastrointestinal tract, liver, and spleen. Recent studies revealed striking differences between the molecular and cellular biology of mast cells in patients with mastocytosis and those of healthy individuals. These findings are being used in formulating diagnostic criteria as well as designing novel treatment approaches to the disease.

摘要

系统性肥大细胞增多症是一种肥大细胞及其祖细胞的克隆性疾病。系统性肥大细胞增多症的症状是由于肥大细胞在骨髓、皮肤、胃肠道、肝脏和脾脏等各种组织中的病理性积聚和激活所致。最近的研究揭示了肥大细胞增多症患者与健康个体的肥大细胞在分子和细胞生物学方面存在显著差异。这些发现正被用于制定诊断标准以及设计针对该疾病的新治疗方法。