摘要
噬血细胞综合征又称噬血细胞性淋巴组织细胞增生症(HLH), 是一种由遗传因素或获得性免疫调节异常导致的过度炎症反应综合征。淋巴瘤相关噬血细胞综合征(LAHS)是继发于淋巴瘤的恶性肿瘤相关性HLH, 其临床误诊率和病死率高, 预后差。现就儿童LAHS的发病机制、诊断及治疗研究进展进行综述, 旨在进一步提高临床医师对儿童LAHS的认识。
Hemophagocytic syndrome,also known as hemophagocytic lymphohistiocytosis(HLH),is a highly stimulated and defective inflammatory response caused by genetic inheritance or acquired immune regulation abnormalities.Lymphoma-associated hemophagocytic syndrome(LAHS)is a malignancy-associated HLH secondary to lymphoma,with a high clinical misdiagnosis rate and fatality rate and poor prognosis.In this article,the pathogenesis,diagnosis and treatment of LAHS in children were reviewed,in order to increase clinician′s understanding of the disease.
作者
高慧霞
李志刚
张蕊
王天有
Gao Huixia;Li Zhigang;Zhang Rui;Wang Tianyou(Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics Key Laboratory of Major Diseases in Children,Ministry of Education Hematology Oncology Center,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China;Laboratory of Hematologic Diseases,Beijing Pediatric Research Institute,Beijing Children′s Hospital,Capital Medical University,National Center for Children′s Health,Beijing 100045,China)
出处
《中华实用儿科临床杂志》
CAS
CSCD
北大核心
2022年第18期1426-1428,共3页
Chinese Journal of Applied Clinical Pediatrics
基金
国家科技重大专项(2017ZX09304029004)
北京市医院管理中心儿科学科协同发展中心专项经费资助(XTZD20180202)
北京市医院管理中心"青苗"计划专项经费资助(QML20181205)
北京市科技委员会首都临床特色应用研究与成果推广(Z171100001017050)
首都卫生发展科研专项研究重点公关项目(2020-1-2022)。
