Shao Xiaoyan, Xu Ye, Xu Xihui, Xu Yong, Chen Hu, Hong Ming, Liu Lingxiang
Department of Hematology, Affiliated Drum Tower Hospital of Nanjing University Medical School, Nanjing, China.
Department of Oncology, First Affiliated Hospital of Nanjing Medical University, Nanjing, China.
Isr Med Assoc J. 2018 Feb;20(2):80-85.
Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disease with poor prognosis despite intensive therapy.
To discuss the ideal therapy of EBV-associated HLH for adults.
We retrospectively studied 23 adult patients with EBV-associated HLH at our institution between January 2000 and June 2015. The clinical characteristics, treatment, and prognosis of adult EBV-associated HLH were analyzed. The median age was 38 years (range 18-72).
All patients were found to have high fever, thrombocytopenia, abnormal liver function, elevated ferritin, and lactate dehydrogenase. Leukopenia, anemia, coagulopathy, hypofibrinogenemia, and splenomegaly were found in more than 80% of patients. Ten patients were treated with HLH-2004 protocol. Eventually, 95.7% of patients died of EBV-associated HLH. Non-HLH-2004 treatment and bone marrow suppression may predict early relapse independently, and the poor performance status and high lactate dehydrogenase level can be poor prognostic factors. It was also validated in comprehensive analysis of published articles.
Adult EBV-associated HLH occurs most often in people of Asian descent who are older than 35 years. These patients had a disappointing outcome despite intensive treatment, especially with high lactate dehydrogenase levels, poor performance status, and bone marrow suppression. HLH-2004 protocol has shown a glimmer of hope in the adult populations.
尽管进行了强化治疗，爱泼斯坦-巴尔病毒（EBV）相关噬血细胞性淋巴组织细胞增生症（HLH）仍是一种罕见的、危及生命且预后不良的疾病。
探讨成人EBV相关HLH的理想治疗方法。
我们回顾性研究了2000年1月至2015年6月在我院就诊的23例成人EBV相关HLH患者。分析了成人EBV相关HLH的临床特征、治疗及预后情况。中位年龄为38岁（范围18 - 72岁）。
所有患者均出现高热、血小板减少、肝功能异常、铁蛋白升高及乳酸脱氢酶升高。超过80%的患者出现白细胞减少、贫血、凝血功能障碍、纤维蛋白原血症降低及脾肿大。10例患者接受HLH - 2004方案治疗。最终，95.7%的患者死于EBV相关HLH。非HLH - 2004治疗及骨髓抑制可能独立预测早期复发，而体能状态差及乳酸脱氢酶水平高可能是不良预后因素。这在对已发表文章的综合分析中也得到了验证。
成人EBV相关HLH最常发生于35岁以上的亚裔人群。尽管进行了强化治疗，这些患者的预后仍令人失望，尤其是乳酸脱氢酶水平高、体能状态差及骨髓抑制的患者。HLH - 2004方案在成人患者中显示出了一线希望。
