以急性肝功能衰竭为表现的噬血细胞综合征临床特点分析

Clinical features of patients with hemophagocytic lymphohistiocytosis presenting as acute liver failure

目的探讨以急性肝功能衰竭为表现的噬血细胞性淋巴组织细胞增多症(HLH)的临床特点。方法对14例以急性肝功能衰竭为表现的HLH患者临床资料进行分析,了解其病因、临床特征、肝功能损害的特点及预后,并总结病因和预后的关系。结果 HLH伴发的急性肝功能衰竭病因多样,以非嗜肝病毒、细菌和真菌感染为主,临床表现以发热和肝脾肿大为主,伴有造血系统、肾脏、心脏等多系统损害,肝功能损害的特征除伴有ALT、AST异常,高胆红素血症和低蛋白血症外,常伴有α-羟丁酸脱氢酶(HBDH)、甘油三酯(TG)、铁蛋白(Ferritin)和乳酸脱氢酶(LDH)的升高。LDH、HBDH增高及多器官功能衰竭(MODF)与预后不良具有独立的相关性(P<0.05)。结论 HLH临床诊断困难,常以急性肝功能衰竭就诊,其肝功能损害的特点有其特殊性,患者预后与病因及肝功能损害程度相关。

Objective To summarize the clinical features of patients with hemophagocytic lymphohistiocytosis （HLH）,but presenting as acute liver failure. Methods Fourteen cases of HLH were included in our study. The etiology,clinical manifestation,hepatic chemical parameters and the relationship between chemical parameters and prognosis of patients were collected. Results Many factors could lead to HLH including viral,bacterial and fungal infection,lymphoma and other unknown reasons in this study. The liver damage of patients were manifested with abnormality of serum alanine transarninase （ALT）,aspirate aminotransferase （AST）,bilirubin,albumin,hydroxybutyrate dehydrogenase （HBDH）,triglyceridemia （TG）,ferritin and lactate dehydrogenase （LDH）.The aberrant LDH,HBDH levels and multiple organ dysfunction failure（ MODF） indicated poor prognosis of these patients independently （P〈0.01）. Conclusion These HLH patients were often presented as acute liver failure,and the severity of liver injury of these patients is related with worse prognosis.