[Clinical Characteristics and Prognostic Analysis of 59 Adult Patients with Epstein-Barr Virus-Associated Hemophagocytic Syndrome].

OBJECTIVE: To analyze the clinical characteristics and the prognostic risk factors of adult patients with Epestein-Barr virus-associated hemophagocytic syndrome (EBV-HLH) so as to enhance the understanding of EBV-HLH and diagnosis and treatment level. METHODS: The clinical manifestation and survival data of 59 adult patients with EBV-HLH admitted in our hospital from January 2013 to August 2018 were analyzed retrospectively. RESULTS: The most common clinical manifestations of 59 patients were high fever (100%), liver dysfunction (91.5%), however the skin rashes (1.7%), and neurologic abnormality (3.4%) were rare. 96.6% of the patients showed the elevation of serum ferritin and LDH level, and hypoproteinemia and sCD25>/=2 400 U/ml were found in 93.2% and 92.3% of the patients, respectively. The median survival time of 59 patients was 2.5+/-0.7 months; overall survival rate of 1, 3, 6 and 12-month was 69.5%+/-6.0%, 44.7%+/-6.6%, 23.9%+/-5.8%, 19.7%+/-5.5%, respectively. Univariate survival analysis showed that the patients with EBV-DNA copies>/=5x10(5)/ml (P0.05), LDH level>/=600 U/L (P0.05) and Plt count20x10(9)/L (P0.05) had poor prognosis, and there was statistically difference in the overall survival rate (P0.01) between HLH-94/2004 group and the group treated without etoposide (not HLH-94/2004). Multivariate analysis revealed that LDH level>/=600 U/L (P0.05), Plt count20x10(9)/L (P0.05) and treatment protocol (not HLH-94/2004) (P0.01) were independent prognostic risk factors in 59 patients with EBV-HLH. CONCLUSIONS: EBV-HLH assocites with severe clinical features, high mortality rate and poor prognosis of patients. EBV-DNA copies>/=5x10(5)/ml (P0.05), LDH level>/=600 U/L (P0.05) and Plt count20x10(9)/L (P0.05) are the poor prognostic factors, and the treatment with HLH-94/2004 protocol can effectively improve the survival of EBV-HLH patients, should be applied as early as possible.