伴有成人噬血细胞综合征的肝衰竭系统分析

Systematic analysis of liver failure accompanied with hemophagocytic syndrome in adults

目的回顾性分析文献报道, 总结伴有成人噬血细胞综合征(HPS)的肝衰竭患者的临床特征。方法通过检索万方、中国知网、维普、PubMed和Ovid数据库, 检索1980年1月至2022年5月发表的伴有成人HPS的肝衰竭患者相关文献, 经筛选后对最终纳入的文献进行系统综述。结果共有77例伴有成人HPS的肝衰竭患者, 平均年龄(41.8±12.5)岁。病例临床表现以持续高热、乏力、消化道症状、重度黄疸最为常见。影像学改变并非特异, 最常见的为肝脾肿大。实验室检查血清总胆红素显著升高, 乳酸脱氢酶、甘油三酯、血清可溶性白细胞介素2受体水平升高, 血清铁蛋白显著升高, 凝血酶原活动度下降, 自然杀伤细胞活性降低, 外周血两系及以上血细胞减少, 骨髓象检查可见噬血细胞。继发于感染的成人HPS更易伴发肝衰竭(55.8%), 总病死率为84.4%。结论伴有成人HPS的肝衰竭患者病死率极高, 预后极差, 早期诊治, 早期治疗是改善预后的关键。

Objective To retrospectively analyze literature reports and summarize the clinical characteristics of liver failure in patients accompanied with adult hemophagocytic syndrome(HPS).Methods The Wanfang,CNKI,VIP,PubMed,and Ovid databases were searched for relevant literature on liver failure patients accompanied with adult HPS published from January 1980 to May 2022.The final included literature was systematically reviewed after screening.Results There were a total of 77 liver failure cases accompanied with adult HPS,with an average age of(41.8±12.5)years.The most common clinical manifestations of these cases were persistent high fever,fatigue,gastrointestinal symptoms,and severe jaundice.The imaging changes were not specific,and the most common was hepatosplenomegaly.Laboratory examination showed a significant increase in serum total bilirubin,lactate dehydrogenase,triglycerides,and soluble interleukin-2 receptor levels,as well as serum ferritin,while there was a decrease in prothrombin activity,natural killer cell activity,and hemocytopenia of two or more lineages in peripheral blood.Myelogram examination showed hemophagocytosis.Adult HPS secondary to infection was more likely to be accompanied with liver failure(55.8%),with an overall case fatality rate of 84.4%.Conclusion Patients with liver failure accompanied with adult HPS have an extremely high mortality rate and a poor prognosis.Early diagnosis and treatment are thekeys to improving theprognosis.