噬血细胞性淋巴组织细胞增多症治疗的临床研究新进展  

Advances in the clinical treatment of hemophagocytic lymphohistiocytosis

作　　者：王昭[1] WANG Zhao(Department of Hematology, Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院血液科

出　　处：《山东大学学报（医学版）》2019年第7期44-49,共6页Journal of Shandong University:Health Sciences

基　　金：国家自然科学基金(81871633);北京市自然科学基金(7172058);北京市医管局扬帆计划重点专业(ZYLX201702);北京市医管局扬帆计划临床创新项目(XMLX201803);北京市医管局登峰人才培养计划

摘　　要：噬血细胞性淋巴组织细胞增多症(HLH)是一种由遗传性或获得性免疫功能异常导致的、以病理性炎症反应为主要特征的高病死率疾病。由国际组织细胞协会制定的HLH-94治疗方案将这一致命性疾病的临床缓解率由过去的不足10%提高到50%~70%,成为目前推荐的一线治疗方案。但是,HLH依然是一种难治性疾病,关于HLH治疗的临床试验在近10年蓬勃开展。这些新的治疗手段包括改进的化学免疫治疗方案,以及新的细胞因子生物靶向治疗等。综述了目前全球范围内关于HLH治疗新方法的临床研究进展,为噬血细胞综合征治疗提供新观点和新思路。Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening disease resulted from hereditary or acquired dysfunction of immune system characterized by pathologic inflammatory reaction. The therapeutic guideline for HLH published by the Histiocyte Society in 1994 was a milestone in HLH treatment, which has improved the clinical remission rate of HLH from less than 10% to 50%-70% and has become a first-line treatment. However, HLH is still a refractory disease, for which numerous clinical trials have been conducted in the past decade. The novel approaches of treatment include modified chemotherapy regimens and biologically targeted therapy. This paper reviews the recent advances in treatment of HLH in order to provide new insights.

关 键 词：噬血细胞性淋巴组织细胞增多症 临床试验 化学免疫治疗 生物靶向治疗补充 

分 类 号：R733.4[医药卫生—肿瘤]