Department of Beijing Pediatric Research Institute, Beijing Children's Hospital, The Capital Medical University, Beijing, China.
Eur Rev Med Pharmacol Sci. 2012 May;16(5):672-8.
BACKGROUND AND OBJECTIVES: Although immunochemotherapy had been reported to be effective initial treatment for patients with Epstein Barr virus-associated hemophagocytic lymphohistiocytosis (EBV-HLH), and stem cell transplantation (SCT) was employed for patients with refractory disease, the long-term outcome of these patients underwent such treatment remained uncertain. The main purpose of this study was to make a primary system review on the outcome of EBV-HLH patients treated with immunochemotherapy and/or SCT. MATERIAL AND METHODS: A system review and meta analysis was conducted on studies which collected from published PubMed and China Knowledge Resource Integrated Database (CNKI). The analysis was based on clinical characteristics and follow-up. Search strategy and selection criteria were identified by relevant articles, the period was defined from January 1990 to October 2010. Search terms included all relevant terms. English and Chinese language papers were reviewed. RESULTS: A total of 11 articles include 342 EBV-HLH patients that were identified with our search terms fulfilled the eligibility criteria. Overall 104/342 patients (30.4%) died at the end of respective study. In 288 patients who did not receive SCT, 93/288 patients (32.3%) patients died. While in 54 patients who underwent SCT, 11/54 patients (20.4%) died at the end of respective study. Four articles had the contents both of immunochemotherapy and SCT. When using a meta analysis compared the mortality between immunochemotherapy and SCT groups, there was no statistical significance could be found, the Odds Ratio is 1.10 (0.43-2.84), (p = 0.84). When compared the mortality between SCT group and total EBV-HLH patients, there was still no statistical significance could be found, the Odds Ratio is 0.99 (0.39-2.53), (p = 0.98). CONCLUSION: Etoposide-containing immunochemotherapy and SCT both decreased the mortality in EBV-HLH patients in the past decade. There was not enough evidence to suggest that SCT is better than immunochemotherapy in children with EBV-HLH. And such result may justify further research.
背景与目的：虽然免疫化学疗法已被报道为 EBV 相关噬血细胞性淋巴组织细胞增多症（EBV-HLH）患者的有效初始治疗方法，且对于难治性疾病患者采用了干细胞移植（SCT），但这些患者接受此类治疗的长期预后仍不确定。本研究的主要目的是对接受免疫化学疗法和/或 SCT 治疗的 EBV-HLH 患者的结局进行初步系统综述。
材料与方法：对发表于 PubMed 和中国知识资源总库（CNKI）的研究进行了系统回顾和荟萃分析。分析基于临床特征和随访情况。通过相关文章确定了检索策略和选择标准，检索时间定义为 1990 年 1 月至 2010 年 10 月。检索词包括所有相关术语。对英文和中文文献进行了综述。
结果：通过我们的检索词共确定了 11 篇文章，其中包含 342 例 EBV-HLH 患者，符合纳入标准。在各自研究结束时，共有 104/342 例（30.4%）患者死亡。在未接受 SCT 的 288 例患者中，93/288 例（32.3%）患者死亡。而在接受 SCT 的 54 例患者中，11/54 例（20.4%）患者死亡。有 4 篇文章同时包含了免疫化学疗法和 SCT 的内容。使用荟萃分析比较免疫化学疗法和 SCT 组之间的死亡率时，无统计学意义，比值比为 1.10（0.43-2.84），（p = 0.84）。比较 SCT 组与所有 EBV-HLH 患者之间的死亡率时，仍无统计学意义，比值比为 0.99（0.39-2.53），（p = 0.98）。
结论：在过去十年中，依托泊苷为基础的免疫化学疗法和 SCT 均降低了 EBV-HLH 患者的死亡率。目前尚无足够证据表明 SCT 优于儿童 EBV-HLH 患者的免疫化学疗法。该结果可能需要进一步研究。
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