Abstract: 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.

Key words: varicella-zoster virus, hemophagocytic hyndrome, clinical features