高细胞因子血症在儿童噬血细胞综合征中的研究进展

The progess of the hypercytokinemia in the children with hemophagocytic syndrome

噬血细胞淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis，HLH）是一组病因错综复杂的，以淋巴细胞和组织细胞反应性活化增生为特征，进而产生大量炎性细胞因子，导致高细胞因子血症和各重要脏器功能损伤的疾病。该疾病包括原发性和继发性两种类型，在疾病的发生发展过程中，高细胞因子血症起到关键性作用，笔者就高细胞因子血症在儿童HLH中的作用进行简要阐述。

Hemophagocytic lymphohistiocytosis （HLH） is a disease caused by various pathogenic factors and characterised by activated lymphoid cells and tissue cells.,which release massive cytokines and results in hypercytokinemia and severe functional distraction of visceral organs. The disease is classified to primary/inherited HPS and secondary/reactive HPS. The hypercytokinemia play a key role in the process of disease development. In this article, the hypercytokinemia in the pathogenesis of children HLH was reviewed.