Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
Requirement for etoposide in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
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DOI：
10.1200/jco.2001.19.10.2665
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发表时间：
2001-05-15
影响因子：
45.3
通讯作者：
Hibi, S
中科院分区：
文献类型：
--
作者：
Imashuku, S;Kuriyama, K;Hibi, S
目的：我们试图确定对成功治疗EB病毒（EBV）相关的噬血细胞性淋巴组织细胞增生症（HLH）最关键的临床变量。测试的因素包括诊断时的年龄（< 2年或< 2年），从诊断到开始使用或不使用含依托泊苷方案治疗的时间，诱导治疗期间环孢菌素A（CSA）给药的时间，结果：Kaplan-Meier分析显示，47例患者（其中大多数为中重度至重度疾病）的整个队列的4年总生存率为78.3% +/- 6.7%（SE）。在确诊后4周内开始依托泊苷治疗的患者长期生存率明显较高（90.2% +/-6.9%vs56.5%+/- 12.6%）。考克斯比例风险模型的多变量分析表明，从EBV-HLH诊断到依托泊苷给药的短时间间隔具有独立的预后意义（缺乏该特征的患者的相对死亡风险为14.1; 95%置信区间为1.16 - 166.7; P = 0.04）。在考克斯模型中，分析的竞争变量均无显著预测强度。然而，伴随使用的CSA与依托泊苷在一个子集的患者似乎已经防止了严重的并发症，从中性粒细胞减少症在第一年的treatment.Conclusion：我们的结论是，早期管理的依托泊苷，最好与CSA，是治疗的选择与EBV-HLH患者。(C)2001年，美国临床肿瘤学会。
Purpose: We sought to identify the clinical variables most critical to successful treatment of Epstein-Barr virus (EBV)-associated hemophagocytic lymphohistiocytosis (HLH).Patients and Methods: Among the factors tested were age at diagnosis (< 2 years or < 2 years), time from diagnosis to initiation of treatment with or without etoposide-containing regimens, timing of cyclosporin A (CSA) administration during induction therapy, and the presence or absence of etoposide.Results: By Kaplan-Meier analysis, the overall survival rate for the entire cohort of 47 patients, most of whom had moderately severe to severe disease, was 78.3% +/- 6.7% (SE) at 4 years. The probability of longterm survival was significantly higher when etoposide treatment was begun less than 4 weeks from diagnosis (90.2% +/- 6.9% v 56.5% +/- 12.6% for patients receiving this agent later or not at all; P < .01, log-rank test). Multivariate analysis with the Cox proportional hazards model demonstrated the independent prognostic significance of a short interval from EBV-HLH diagnosis to etoposide administration (relative risk of death for patients lacking this feature, 14.1; 95% confidence interval, 1.16 to 166.7; P = .04). None of the competing variables analyzed had significant predictive strength in the Cox model. However, concomitant use of CSA with etoposide in a subset of patients appears to have prevented serious complications from neutropenia during the first year of treatment.Conclusion: We conclude that early administration of etoposide, preferably with CSA, is the treatment of choice for patients with EBV-HLH. (C) 2001 by American Society of Clinical Oncology.