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

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。

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

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.