CAJ | 학술논문

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。
목적：연구성인서혈세포종합정（hemophagocytic syndrome, HPS）환자간손상적림상특점。방법대33례성인HPS환자적병인、림상특점급실험실결과진행분석。결과33례중，병인여감염상관17례（51.5%），여비감염상관8례（24.2%），령유8례（24.2%）미조도원발병。16례사망，병사솔위48.5%。소유환자균유불동정도간손상，돌출표현위LDH화AST명현승고，ALB명현하강，TBIL승고이DBIL위주，병반유불동정도적PA하강。단재사망조화존활조간지유TBIL화PA차이유통계학의의[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]。결론간손상시HPS상견적기관손해，간손상정도가능여예후상관。
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.