Chen R L, Lin K H, Lin D T, Su I J, Huang L M, Lee P I, Hseih K H, Lin K S, Lee C Y
Department of Paediatrics, College of Medicine, National Taiwan University, Taipei.
Br J Haematol. 1995 Feb;89(2):282-90. doi: 10.1111/j.1365-2141.1995.tb03302.x.
The Epstein-Barr virus (EBV), or human herpesvirus-6 (HHV-6) associated haemophagocytic lymphohistiocytosis, has been found prevalent in Taiwan; it affects previously healthy children and is always fatal when treated only supportively. Recognition of the underlying pathogenesis for this disease prompted adoption of an immunomodulatory regimen of intravenous immunoglobulin (IVIG) and/or etoposide on 17 such patients treated between 1990 and 1993. Remarkable improvement in patients' prognoses was demonstrated. Eight patients are still alive with a median follow-up of 1 year and 2 months post-treatment. Both IVIG and etoposide had positive immunomodulation effects such as alleviation of fever and normalization of haematological and hepatic parameters. Sustained complete response was obtained in two of nine cases of EBV-associated diseases treated with IVIG only. EBV transcripts became undetectable after etoposide and/or IVIG treatment without antiviral agents. Etoposide given by split-doses schedule appeared to be superior to conventional three-consecutive-days schedule for both remission induction and disease-free survival. Our preliminary trial apparently provides a promising improvement in the treatment of this previously fatal disease. IVIG or etoposide is effective in reversing the process of lymphohistiocytic dysregulation resulting from virus infection of immune cells in this syndrome and probably helps hosts to control active virus replication in certain cases, through immunomodulation.
爱泼斯坦-巴尔病毒（EBV）或人类疱疹病毒6型（HHV-6）相关噬血细胞性淋巴组织细胞增生症在台湾地区较为普遍；该疾病影响既往健康的儿童，若仅给予支持性治疗往往会致命。对该疾病潜在发病机制的认识促使对1990年至1993年间治疗的17例此类患者采用静脉注射免疫球蛋白（IVIG）和/或依托泊苷的免疫调节方案。结果显示患者的预后有显著改善。8例患者仍存活，治疗后中位随访时间为1年零2个月。IVIG和依托泊苷均具有积极的免疫调节作用，如缓解发热以及使血液学和肝脏参数恢复正常。仅接受IVIG治疗的9例EBV相关疾病患者中有2例获得持续完全缓解。在未使用抗病毒药物的情况下，依托泊苷和/或IVIG治疗后EBV转录本检测不到。对于诱导缓解和无病生存期，采用分剂量给药方案的依托泊苷似乎优于传统的连续三天给药方案。我们的初步试验显然为这种既往致命疾病的治疗带来了有希望的改善。IVIG或依托泊苷可有效逆转该综合征中免疫细胞病毒感染导致的淋巴组织细胞失调过程，并且在某些情况下可能通过免疫调节帮助宿主控制活跃的病毒复制。
