摘要
噬血细胞性淋巴组织细胞增生症(HLH),即噬血细胞综合征(HPS),主要分为原发性HLH与继发性HLH,前者主要由HLH相关基因突变导致,后者主要由感染、肿瘤与自身免疫性疾病触发。HLH的主要特征为在各种致病因素的作用下,机体免疫功能紊乱引起单核/巨噬细胞系统反应性增生,释放大量γ干扰素、肿瘤坏死因子(TNF)-α等细胞因子,导致多器官、系统功能受损,并且进行性加重。HLH患者的主要临床表现为发热,肝、脾大与全血细胞减少,亦可表现为血清铁蛋白水平升高、高甘油三脂血症、肝功能异常、低纤维蛋白原血症及神经系统症状等。HLH是一种临床表现复杂、疾病进展迅速及病死率高的疾病,目前其发病机制与治疗方法尚在探索中。为了进一步了解HLH相关研究进展,并为其治疗方法的完善提供基础,笔者拟就近年HLH发病机制的研究现状进行介绍。
Hemophagocytic lymphohistiocytosis(HLH)is also known as hemophagocytic syndrome(HPS).It is mainly divided into primary HLH and secondary HLH.The former is mainly associated with related gene mutations,while the latter is mainly triggered by infection,tumor and autoimmune diseases.The main characteristic of HLH is that the immune dysfunction leads to reactive proliferation of monocytes/macrophages and the release of large amounts of cytokines,such as interferon-γand tumor necrosis factor(TNF)-αunder various pathogenic factors,leading to impaired and progressive aggravation of multi-organ and systems.The main clinical manifestations of HLH patients are fever,hepatosplenomegaly and pancytopenia,as well as serum ferritin elevation,hypertriglyceridemia,liver dysfunction,low fibrinogenemia and neurological symptoms etc..HLH is a disease with complex clinical manifestation,rapid disease progression and high fatality rate.At present,the treatment methods are being explored,and better treatment methods can be found only by understanding the pathogenesis of HLH.To further understand the research progress of this disease,and to provide the basis for the improvement of treatment methods,this article summarizes the research status on the pathogenesis of HLH in recent years.
作者
韩迎弟
王颖韶
白洁
Han Yingdi;Wang Yingshao;Bai Jie(Department of Hematology,Second Hospital of Tianjin Medical University,Tianjin 300211,China)
出处
《国际输血及血液学杂志》
CAS
2020年第3期222-228,共7页
International Journal of Blood Transfusion and Hematology
基金
国家自然科学基金(81170128)。
