“Death is coming”:噬血细胞综合征合并出凝血功能障碍  

Death is coming:hemophagocytic lymphohistiocytosis complicated by coagulation disorders

作　　者：童文佳 金丹群 Tong Wenjia;Jin Danqun(Intensive Care Unit,Anhui Provincial Children's Hospital,Hefei 230051,China)

机构地区：[1]安徽省儿童医院重症医学科,合肥230051

出　　处：《中国小儿急救医学》2024年第11期825-830,共6页Chinese Pediatric Emergency Medicine

基　　金：安徽省自然科学基金面上项目(2308085MH267)。

摘　　要：噬血细胞性淋巴组织细胞增多症(HLH)是一种病情复杂、进展迅速且高致死性疾病,可引起多器官功能障碍。其中,出凝血功能障碍可贯穿于HLH的整个病程,是导致早期死亡的重要因素。对于此类患者,早期诊断凝血功能障碍或弥散性血管内凝血(DIC)非常具有挑战性,因为HLH本身会干扰凝血功能障碍或DIC的诊断指标。本文总结了国内外研究现状,探讨了HLH合并凝血功能障碍的概念、发病机制以及常用和新型凝血指标,协助判断HLH合并凝血功能障碍或DIC的发生及预后,以期为原发病的后续治疗提供更多机会。Hemophagocytic lymphohistiocytosis(HLH)is a complex,rapidly progressing,and highly fatal disease that can lead to during multiple organ dysfunctions.Coagulation disorders frequently occur during the course of HLH and are a significant cause of early mortality.Early diagnosis of coagulation disorders or disseminated intravascular coagulation(DIC)in such patients is particularly challenging,as HLH itself interferes with the diagnostic markers associated with coagulation disorders or DIC.This article summarized the current state of research on HLH complicated by coagulation disorders,including its pathogenesis and the common and novel coagulation markers used in the collaborative assessment of HLH complicated by coagulation disorders or DIC.The goal was to provide more opportunities for subsequent treatment of the underlying disease.

关 键 词：噬血细胞综合征 凝血功能障碍 弥散性血管内凝血 噬血细胞性淋巴组织细胞增多症 

分 类 号：R593[医药卫生—内科学]