肝功能异常的嗜血细胞综合征临床和实验室特点

Clinical and laboratory characteristics of hemophagocytic syndrome in patients with liver dysfunction

目的分析肝功能异常的嗜血细胞综合征患者的临床和实验室特点,提高对该病的认识。方法回顾调查患者的临床基本情况、原发病、实验室结果、治疗和预后;全自动生化分析仪检测患者肝功能结果,全血细胞分析仪检测血常规;骨髓穿刺进行骨髓检查分析。结果 7例患者均有不同程度的肝脾肿大、发热、肝功能异常及血细胞减少,6例低纤维蛋白原血症,4例自然杀伤细胞活性降低。骨髓涂片6例见吞噬血细胞现象;2例患者死亡,5例患者放弃治疗。结论临床应重视肝功能异常、持续发热且抗生素治疗无效的嗜血细胞综合征患者的诊治。

Objective To analyze the clinical and laboratory characteristics of hemophagocytic syndrome in patients with liver dysfunction. Methods Clinical data of 7 patients including demographic characteristics, underlying disease, laboratory findings, treatment and clinical outcome, were collected in a retrospective survey. The liver function test were detected by automatic biochemical analyzer., Marrow biopsy was done at the same time. Results All 7 patients had fever and hepatosplenomegaly, Laboratory data mairily manifested with liver dysfunction（7/7）, cytopenia（7/7）, hypofibrinogenemia （6/7）, lower NK-cell activity（4/7）and hemophagocytosis of bone marrow smears （6/7）. Two patients were died. Five patients abandoned treatment. Conclusion Clinicians need to be alert and suspect this entity in patients with hemophagocyt- ic syndrome with characteristics of liver dysfunction and high fever that does not responding to broad-spectrum antibiotics.