肝移植术后嗜血细胞综合征的初步观察

目的 观察肝移植术后嗜血细胞综合征（HPS）的临床表现、诊断、治疗及结果。方法 对4例肝移植术后HPS的临床表现如发热、皮疹、肝、脾、淋巴结肿大，实验室检查如全血细胞减少、肝功能异常、高三酰甘油血症、低纤维蛋白原血症、铁蛋白异常、骨髓检查进行总结分析。结果2例患者分别于术后57、24d死亡，死亡原因为感染及多器官功能衰竭。2例经对症治疗后恢复健康。结论 肝移植术后发生全血细胞减少，要警惕HPS的发生，应及时检查骨髓及做相应的检查。

The preliminary observation on hemophagocytic syndrome after liver transplantation

Objective To summarize the clinical experience of diagnosis and treatmemt about hemophagocytic syndrome after liver trans- plantation. Methods The clinical presentation such as fever,hepatosplenomegaly,skin rash,liver dysfunction and laboratory examination results such as paneytopenia,fibrinogen,hypertriglyceridemia,ferritin and bone marrow examination were observed in 4 patients. Results The first and third patient died from infection and multiple organ failure on the 57th and 24th day after transplantation,respectively The second and fourth patients recovered. Conclusion When pancytopenia was observed after liver transplantation,hemophagoeytic syndrome should be suspected,and bone marrow examination and the others should be carried out .