成人噬血细胞综合征肝损伤临床特点分析

Clinical features of liver injury in adult patients with hemophagocytic syndrome

目的研究成人噬血细胞综合征(hemophagocytic syndrome,HPS)患者肝损伤的临床特点。方法对33例成人HPS患者的病因、临床特点及实验室结果进行分析。结果 33例中,病因与感染相关17例(51.5%),与非感染相关8例(24.2%),另有8例(24.2%)未找到原发病。16例死亡,病死率为48.5%。所有患者均有不同程度肝损伤,突出表现为LDH和AST明显升高,ALB明显下降,TBIL升高以DBIL为主,并伴有不同程度的PA下降。但在死亡组和存活组间只有TBIL和PA差异有统计学意义[TBIL(307.3±182.2)μmol/L vs(137.0±153.7)μmol/L,P=0.013;PA(44.5±18.8)%vs(64.9±19.1)%,P=0.004]。结论肝损伤是HPS常见的器官损害,肝损伤程度可能与预后相关。

Objective To investigate the clinical features of liver injury in adult patients with hemophagocytic syndrome （HPS）. Methods The etiology, clinical features and laboratory findings of 33 adult patients with HPS were analyzed retrospec-tively. Results Among 33 patients, the etiology related to infections was found in 17 （51.5%） patients, the etiology related to non-infections was found in 8 （24.2%） patients, and no underlying disease was found in 8 （24.2%） patients. Sixteen patients died, with the mortality rate of 48.5%. Different degrees of liver injury were observed in all the patients, manifested by an obvious in-crease in the activity of LDH and AST level and an obvious decline in ALB level, as well as an increase in TBIL level, mainly in DBIL, and a different degree of decrease in PA. However, only TBIL and PA were significantly different between the non-sur-vivor group and the survivor group （TBIL 307.3±182.2μmol/L vs 137.0±153.7μmol/L, P=0.013;PA 44.5%±18.8%vs 64.9%±19.1%, P=0.004）. Conclusions Liver injury is a common organ damage in HPS patients, and the degree of liver injury may be related to the prognosis.