15例嗜血细胞综合征合并肝损伤的临床分析

目的 分析嗜血细胞综合征(HPS)合并肝损伤的临床特点.方法 对我院2006年1月至2010年12月就诊的15例HPS合并肝损伤患者的病因、临床特点及转归进行分析.结果 本组病例发病以持续高热(100.0%)、浆膜腔积液(80.0%)、黄疸(80.0%)、脾肿大(73.3%)、肝肿大(66.7%)、淋巴结肿大(60.0...

Conical analysis of 15 patients with hemophagocytic syndrome

Objective To investigate the clinical features of hemophagocytic syndrome(HPS) with liver injury. Methods To analyze the clinical characteristics and outcomes in 15 patients with HPS, who enrolled in our hospital fron January 2006 to Deccmber 2010. Results HPS was clinically characterized by prolonged high-grade fever (100.0%), hydrops of multiple serous cavity (80.0%), jaundice (80.0%), splenomegaly (73.3%), hepatomegaly (66.7%), and lymphadenopathy (60.0% ), and other features including central nervous system abnormality (26.7%) and alimentary tract hemorrage(20.0%). Laboratory data indicated that liver injury was the most prominent feature (100.0%), mainly manifested with dramatically increased level of total bilirubin, AST, ALT, γ-GT and ALP, and with decreased level of CHE and ALB. All the patients were with hemophagocytosis in bone marrow and high level of ferritin, some were with peripheral cytopenias in two or more lineages (93.3%), hypertriglyceridemia (70.0%), and hypofibrinogenemia (64.3% ). High concentration of serum soluble CD25 and low NK cell activity could be found in 3 patients who got the tests. The disease with infection was most common (53.3%). Systemic lupus erythematosus and lymphoma were also the causes of HPS (6.7%). Nine patientis were dead and the mortality was 60.0% . High dose of methylprednisolone and gamma globulin at the early phase of HPS could improve the outcome. Conclusions There are various underlying disease and clinical manifestations of HPS. Prolonged high-grade fever and liver dysfunction are the prominent features of HPS. HPS is a severe disorder with poor prognosis.