Objective: To explore the clinical feature of liver injury in patients with hemophagocytic syndrome (HPS).
Methods: The clinical data of 92 patients with HPS in our hospital were analyzed retrospectively, and the characteristics of hepatic lesion and its relationship with prognosis in HPS patients were explored.
Result: 92 cases of HPS showed different degrees of liver dysfunction from mild to moderate. The clinical parameters of liver dysfunction included the increased level of LDH (89.13%), AST (64.13%), TBIL (59.78%) and decreased level of ALB (90.22%). Moreover, 76.09% and 67.39% of the patients had the prolonging of APTT and PT respectively. The ALB level of patients in rheumatoid immune group were higher than that in infection, maglinancy and unexplained groups, all with statistically and significant difference (P＜0.05, P＜0.05 and P＜0.01), the ALB level of patients in infection group were statistically and significantly higher than that in unexplained group (P＜0.01). The Fbg level of patients in infection group were lower than that in maglinancy group, unexplained group and rheumatoid immune group, all the differences were statistically significant (P＜0.05, P＜0.01 and P＜0.05). Child-Pugh grading was further carried out in HPS patients with liver disfunction. Survival time of the patients grade A was significantly higher than that of grade B and C of patients. Univariate analysis showed that the patients with LDH≥2000 U/L, ALB＜30 g/L and PT≥15.1 s had a survival time inferior to control patients (P＜0.05, P＜0.01 and P＜0.01, respectively). Multivariate analysis showed that ALB＜30 g/L was an independent adverse prognostic factor for these patients (P＜0.01).
Conclusion: Patients with HPS generally have impaired liver function mainly manifested with elevated LDH and AST levels, and declined ALB level, which may correlate with the disease cause and prognosis. Patients with LDH≥2000 U/L, ALB＜30 g/L and PT≥15.1 s have a poorer prognosis and should be treated as soon as possible.
题目: 噬血细胞综合征患者肝功能损害的临床特点分析.
目的: 研究噬血细胞综合征（hemophagocytic syndrome，HPS）患者肝功能损害的临床特点.
方法: 回顾性分析本院92例HPS患者肝功能损害相关的临床资料，探讨肝功能异常的特点及其和HPS患者生存的关系.
结果: 92例HPS患者均有不同程度的肝功能异常，以轻、中度损害为主。肝功能损害主要表现为乳酸脱氢酶（LDH，89.13％）和天门冬氨酸氨基转氨酶（AST，64.13％）明显升高，白蛋白（ALB，90.22％）显著下降，总胆红素升高以直接胆红素（DBIL，59.78％）为主，伴活化部分凝血活酶时间（APTT，76.09％）以及凝血酶原时间（PT，67.39％）的延长。风湿免疫组患者的ALB水平较感染组、肿瘤组、不明原因组患者均明显增高（P＜0.05、P＜0.05和P＜0.01），感染组患者ALB水平较不明原因组患者明显增高（P=0.007）。感染组患者的Fbg水平较肿瘤组、不明原因组、风湿免疫组患者均明显降低（P＜0.05、P＜0.05和P＜0.05）。将HPS患者进行肝功能Child-Pugh分级，A级组患者的生存率明显高于B级组和C级组。单因素分析显示，LDH≥2 000 U/L、ALB＜30 g/L 和PT≥15.1 s的患者预后更差（P＜0.05、P＜0.01和P＜0.01）；多因素分析显示，ALB＜30 g/L是影响患者生存的独立不良预后因素（P＜0.001）.
结论: HPS患者普遍存在肝功能损害，主要表现为LDH和AST水平升高以及ALB水平降低。肝功能损害程度可能与HPS的病因及预后相关，LDH ≥2 000 U/L、ALB＜30 g/L和PT≥15.1 s的HPS患者预后更差，临床上应尽早给予积极治疗.