病毒相关性噬血细胞综合征的诊断与治疗

目的　提高对病毒相关性噬血细胞综合征的认识。方法　回顾性分析 1 6例病毒相关性噬血细胞综合征患儿的临床资料。结果　临床表现为持续性高热 ,肝脾大 ,血常规二系或三系减少 ;高三酰甘油血症、血清铁蛋白 (SF)增高 ;纤维蛋白原降低 ,凝血功能障碍 ;CD4/CD8下降 ,非杀伤细胞比例降低 ,高细胞因子血症。骨髓中找到噬血细胞。大剂量人血丙种球蛋白、甲泼尼龙治疗后 ,好转 7例 ,死亡 5例 ,4例放弃治疗。结论　早期诊治是提高病毒相关性噬血细胞综合征患儿生存率的关键。

Clinical Analysis of Virus-Associated Hemophagocytic Syndrome in Children

Objective To increase the awareness of virus-associated hemophagocytic syndrome.Methods Sixteen cases of virus-associated hemophagocytic syndrome were retrospectively analyzed.Results All presented with persistent high fever,cytopenia,hepato-splenomegaly,hepatic dysfunction,hypertriglyceridemia,hyperferritinemia,coagulopathy,hypofibrinogenemia,cytokine storm and a low natural killer cell activity.All patients had lymphohistiocytic accumulation in bone marrow.Treatment with high-dose gamma-globulin and high-dose methylprednisolone.Clinical symptoms and laboratory improved,and five patients died.Conclusion Aggressive early diagnosis and treatment are critical to improve survival.