含利妥昔单抗方案治疗EB病毒相关嗜血细胞性淋巴组织细胞增多症六例临床分析

目的：观察含利妥昔单抗方案治疗EB病毒相关嗜血细胞性淋巴组织细胞增多症（EBV-HLH）的效果。方法回顾性分析6例接受含利妥昔单抗方案治疗的EBV-HLH患者的临床资料，随后确诊为淋巴瘤相关及原发性HLH患者未纳入分析。结果6例EBV-HLH患者均为男性，年龄20～61岁，中位年龄27.5岁（21～60岁）。2例初治EBV-HLH患者治疗后2周达到部分缓解，但均在4周内复发；4例复发EBV-HLH患者均未缓解。6例EBV-HLH患者最终均因原发病进展、合并出血或感染等死亡。利妥昔单抗治疗前1周和治疗后1～2周白细胞、血红蛋白、血小板、铁蛋白、丙氨酸氨基转移酶、天冬氨酸氨基转移酶、总胆红素、纤维蛋白原和EBV-DNA共9项指标差异均无统计学意义（均P＞0.05）。结论含利妥昔单抗方案治疗EBV-HLH的疗效不及既往研究结果，有待于前瞻性大规模利妥昔单抗单药治疗EBV-HLH的临床试验进一步验证。

Clinical study of 6 patients with Epstein-Barr virus associated hemophagocytic lymphohistiocytosis treated with rituximab-containing regimen

Objective To investigate the efficacy of rituximab-containing regimen in Epstein-Barr virus associated hemophagocytic lymphohistiocytosis (EBV-HLH). Methods A retrospective analysis involving 6 EBV-HLH patients who had received treatment with rituximab-containing regimen was performed. The patients who were diagnosed with lymphoma or primary HLH subsequently were not included in the analysis. Results All patients were males. The median age was 27.5 years (range 20-61 years). Two patients received rituximab-containing regimen as primary therapy, and got partial remission (PR) within 2 weeks after the first course of rituximab, but relapsed within 4 weeks. Four patients received rituximab-containing regimen as salvage therapy, but none achieved remission. The 6 patients died due to HLH and complications, such as infection and hemorrhage. Laboratory data including white blood cell count, haemoglobin concentration, platelet count ferritin, alanine transaminase, aspartate transaminase,total bilirubin, fibrinogen and EBV-DNA did not show statistical significance (all P>0.05). Conclusion The efficacy of rituximab as a treatment for EBV-HLH is not as good as that in the previous study, and a prospective clinical trial of rituximab-based monotherapy is needed to answer the question.