淋巴细胞变异型嗜酸性粒细胞增多症基础与临床

摘要

Eosinophil disorders are a range of heterogeneous diseases, which manifest as persistent increase of eosinophile granulocyte in blood and tissue, and may cause microenvironment change and irreversible chronic organ damage. According to World Health Organization, eosinophil diseases were divided into hypereosinophilia (HE) and hypereosinophilia syndrome (HES) by whether the organ is involved. Besides familial HE, HE were also divided into secondary (reactive) HE, primary (clonaleoplastic) HE, and idiopathic HE. Lymphocytic variant HE is a special kind of secondary HE. With the study of the molecular mechanism of lymphocytic variant HE, the diagnosis is gradually clear. Moreover, some targeted therapeutic drugs, such as anti IL-5 monoclonal antibody (Mepolizumab) and anti CD 52 monoclonal antibody (Alemtuzumab) were also appeared. In this paper, the current basic and clinical researches of lymphocytic variant HE are reviewed.%嗜酸性粒细胞增多性疾病是一组异质性疾病，表现为血液以及组织中嗜酸性粒细胞持续性增多，并因嗜酸性粒细胞增殖所产生的炎症物质而造成微环境的改变和慢性不可逆的器官损害。世界卫生组织根据有无器官受累，将嗜酸性粒细胞增多性疾病分为嗜酸性粒细胞增多症（HE）和嗜酸性粒细胞增多综合征（HES）。除家族性HE外，又分为继发性（反应性）、原发性（克隆性）和特发性HE。而淋巴细胞变异型HE是继发性HE的一种特殊类型。随着对淋巴细胞变异型HE发生的分子机制的研究，其诊断逐渐明确，并且一些靶向治疗药物如抗IL-5单克隆抗体Mepolizumab、抗CD 52单克隆抗体Alemtuzumab也相继出现。文章综述淋巴细胞变异型HE的基础与临床现况。