Assessment of immunochemotherapy and stem cell transplantation on EBV-associated hemophagocytic lymphohistiocytosis in children: a systematic review and meta analysis

作者：Qin, Q.; Xie, Z.; Shen, Y.; Yang, S.; Liu, C.; Huang, Z.; Xu, J.; Ai, J.; Shen, K.^*

来源：EUROPEAN REVIEW FOR MEDICAL AND PHARMACOLOGICAL SCIENCES, VERDUCI PUBLISHER, VIA GREGORIO VII, ROME, 186-00165, ITALY, 672-678, 2012-05.

• Epstein-Barr virus
• Hemophagocytic lymphohistiocytosis
• Hematopoietic stem cell transplantation
• Meta analysis

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 January1990 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 similar to 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 similar to 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.

• 出版日期2012-5
• 单位首都医科大学