EB病毒感染相关性噬血细胞综合征37例单中心回顾性分析

Retrospective analysis of EB virus-related hemophagocytic syndrome:a single center experience of 37 patients

目的:探讨EB病毒相关性噬血细胞综合征(EBV-HPS)的临床特点、治疗以及预后危险因素。方法:对我院37例EBV-HPS患者的临床表现、实验室检查指标、治疗方案及临床转归进行回顾性分析。结果:发热是最常见的临床表现,实验室检查中乳酸脱氢酶(LDH)、铁蛋白及高密度脂蛋白最敏感。与未成年患者相比,成年患者发病时血小板往往更低,低钠低钾血症发生率也较高,肝脏肿大相对少见。37例患者中完全缓解11例,部分缓解4例,无效18例,总有效率为41%(另外有4例仅给予对症支持治疗)。化疗患者有效率为64.7%,明显高于未化疗患者。患者中位随访时间为34个月,1个月生存率为45%,6个月生存率为31%,12个月生存率为25%。治疗后疾病控制情况及是否应用化疗药物是影响预后的重要因素。在进一步治疗的33例患者中,化疗患者1年总生存率达41.2%,激素及丙种球蛋白患者1年总生存率为10.3%(P=0.03)。白蛋白是影响预后的另一个重要指标,发病时中性粒细胞数量、EBV拷贝数及患者年龄均可能影响预后,而LDH及铁蛋白与预后并无明显相关性。结论:LDH、铁蛋白及高密度脂蛋白是诊断EBV-HPS最敏感的指标,未成年患者及成年患者可能具有不完全相同的临床表现。EBV-HPS预后不佳,一旦确诊需要尽快应用以VP16为主的化疗方案。治疗方案是影响预后的主要因素,LDH及铁蛋白与预后并无明显相关性。

Objective：To investigate clinical features,treatment and prognostic factors of EBV-HPS.Method：A retrospective study was carried out to analyze the clinical features,laboratory findings,treatment and clinical outcomes of 37 patients with EBV-HPS.Result：Fever was the most common clinical manifestation,while lactate dehydrogenase,ferritin and high density lipoprotein were the most sensitive laboratory tests.Compared with the teenagers,the count of platelets in adult patients was often lower.The incidence of low sodium and low potassium was higher than that of adults,liver enlargement was relatively rare.Of the 37 patients studied,11 patients achieved complete remission,4patients achieved partial remission,18 patients were ineffective,and the total efficiency was41%.The efficiency of systemic chemotherapy was 64.7%,which was significantly higher than that of no-chemotherapy.The median follow-up time was 34 months.Overall survival at 1 month,6 months and 12 months was45%,31% and 25%respectively for all the patients.Both the application of chemotherapy drugs and the remission status after initial treatment were important prognostic factors.In 33 patients for further treatment,overall survival at 1years was 41.2%for patients received systemic chemotherapy,and was only 10.3%for those received corticosteroids and intravenous immunoglobulin（P=0.03）.ALB was another important indicator of the prognosis,the number of neutrophils,EBV viral load and the age of patients may affect the prognosis,while LDH and ferritin was not significantly related.Conclusion：LDH,ferritin and high density lipoprotein are the most sensitive diagnostic indicators of EBV-HPS.Teenagers and adult patients may vary in their clinical manifestations.The prognosis of EBV-HPS is poor,once EBV-HPS is diagnosed,etoposide based chemotherapy regimen need to be administrated as soon as possible.Therapeutic regimen is the main factor affecting the prognosis,while LDH and ferritin do not affect prognosis of EBV-HPS.