中国妇幼健康研究2017，Vol.28Issue(3)：357-360,4.DOI:10.3969/j.issn.1673-5293.2017.03.045
儿童噬血细胞综合征发病机制研究进展
Research progress on pathogenesis of children with hemophagocytic syndrome
于程 1李长钢2
作者信息
- 1. 遵义医学院珠海校区,广东珠海519041
- 2. 遵义医学院附属深圳市儿童医院,广东深圳518038
- 折叠
摘要
Abstract
Hemophagocytic syndrome ( HPS) , named hemophagocytic lymphohistiocytosis ( HLH) , is a set of clinical syndrome resulting from various causes of autoimmune system function disorder .Cytotoxic effect of cytotoxic T lymphocytes ( CTL ) and NK cells reduces significantly, and antigen including virus cannot be eliminated timely , and thus CTL and macrophage are abnormally and continuously active and hyperplastic .They are correlated with HPS .The physiopathologic mechanism mainly is due to immune dysfunction and /or immunoregulatory dysfunction , so the autologous lymphocytes and tissue cells activate excessively , which leads to rapid and severe inflammatory response with the clinical features of high fever , hepatosplenomegaly , pancytopenia , abnormal liver function and blood coagulation dysfunction .The pathogenesis of the disease is not yet completely clear .Onset of the disease is quick and it develops rapidly with high mortality and complex manifestations .Children have high incidence of HPS , so it tends to be misdiagnosed or delayed for treatment and thus easily leads to death of children .So this paper reviewed the progress of pathogenesis about HPS in children in recent years.关键词
儿童/噬血细胞综合征/发病机制/进展Key words
children/hemophagoeytic syndrome ( HPS)/pathogenesis/progress分类
医药卫生引用本文复制引用
于程,李长钢..儿童噬血细胞综合征发病机制研究进展[J].中国妇幼健康研究,2017,28(3):357-360,4.
