噬血细胞综合征继发凝血功能障碍的认识与研究

Research on coagulopathy of Hemophagocytic syndrome

噬血细胞综合征(HPS)是淋巴细胞和组织细胞非恶性增生,产生细胞因子风暴所导致的一种临床综合征。临床表现多样化,其中凝血功能障碍与HPS患者高病死率密切相关。作者对噬血细胞综合征患者发生凝血功能障碍的病理生理机制、实验室表现、临床表现及治疗加以综述,以期提高对噬血细胞综合征并发凝血功能障碍的认识,从而及时发现并治疗凝血功能障碍,降低早期病死率,改善预后。

Hemophagocytic syndrome（HPS） is a syndrome caused by the uncontrolled proliferation of activated lymphocytes and histio- cytes secreting high amounts of inflammatory cytokines. The clinical manifestations are various, and the Coagulopathy is related with high mortality rate closely. In this paper,we review the pathogenesis,laboratory findings, clinical features and treatment of coagulopathy on Hemophagocytic Syndrome, and clinical physicians should pay high attention to these to reduce the mortality rate and improve prognosis of HPS.