水痘-带状疱疹病毒相关性噬血细胞综合征一例并文献复习

Varicella-zoster virus-related hemophagocytic syndrome:a case report and literature review

目的:总结水痘-带状疱疹病毒相关性噬血细胞综合征(varicella zoster virus associated hemophagocytic syndrome,VZV-HPS)的临床特点。方法:收集本院1例VZV-HPS患者的病历资料,通过检索相关的国内外文献数据库,筛选并提取17个病例进行分析。结果:18例VZV-HPS患者中男女比例3.5∶1,12例(66.7%)≤17岁。18例患者均出现皮疹和血细胞减少,17例(94.4%)发热,15例(83.3%)查见噬血细胞,13例(72.2%)ALT或Fer升高,11例(61.1%)LDH升高。治疗上主要是应用抗病毒药物、糖皮质激素、人免疫球蛋白。治疗中位周期28(5~56)天。15例患者噬血症状完全缓解,2例死亡,1例放弃治疗。结论:VZV-HPS好发于17岁以下男性,以皮疹、发热、血细胞减少、肝损害为主要表现,病情复杂凶险但治愈率高。

Objective:To summarize the clinical characteristics of varicella zoster virus-associated hemophagocytic syndrome(VZV-HPS).Methods:One VZV-HPS patient in our hospital and 17 cases reported in domestic and foreign database were analyzed.Results:Eighteen patients were enrolled.The male to female ratio was 3.5∶1 and 12 cases(66.7%)were under 17 years old.18 patients had rash and cytopenia,17(94.4%)had fever,15(83.3%)had hemophagocytes,13(72.2%)had high level of ALT or Fer,and 11(61.1%)had high level of LDH.The main treatment included antiviral drugs,glucocorticoid,human immunoglobulin.The median treatment period was 28(5-56)days.15 patients had complete remission of hemophagy,2 died and one gave up treatment.Conclusions:VZV-HPS most occurs in men under 17 years of age,and the main clinical manifestations are rash,fever,cytopenia and liver damage.The disease is complex and dangerous but the cure rate is high.