儿童EB病毒相关噬血淋巴组织细胞增生症26例

Analysis of 26 cases with EBV-associated hemophagocytic lymphohistiocytosis

目的 探讨儿童EB病毒相关噬血淋巴组织细胞增生综合症（EBV-HLH）的病原学特征及临床实验室特点、治疗及预后相关因素.方法 收集2003年8月～2006年8月我院收治的26例EBV-HLH患儿临床及实验室资料,追踪并回顾性分析.结果 本组15例患儿EBV-HLH由重症传染性单核细胞增多症（IM）快速进展所致;8例为既往感染的再激活,3例由慢性活动性EB病毒感染（CAEBV）发展而来.实验室检查：26例患儿有不同程度的外周血三系减低及凝血功能异常并伴有脂质代谢紊乱,骨髓中出现吞噬血细胞现象.治疗及转归：在26例患儿中,16例患儿死亡,总死亡率61.5%.化疗组19例死亡总死亡率47.4%,非化疗组7例死亡率100%,2组有显著性差异（P=0.023）.脏器损害大于4个部位与死亡相关（P=0.009）.在10例存活患儿中,至少9例仍存在EB病毒的活动.结论 EBV-HLH病情凶险,预后差,以多脏器损害者更差.早期诊断并尽早开始化疗可以提高患儿的存活率.缓解后可继续处于CAEBV感染状态下,甚至可转化成EBV相关淋巴瘤.

Objective To study the characteristics of pathogen, clinical and laboratory features, treatment and prognosis of EBV-associated Hemophagocytic lymphohistiocytosis （EBV- HLH）. Methods To review and analyze the clinical and laboratory date of twenty-six patients, with EBV-HLH from August 2003 to August 2006 in our hospital retrospectively. Results （1） 15 cases were developed from IM in short time; 8 cases were undergoing EBV reactivation; 3 cases were progress from CAEBV. （2） Main clinical features： persistent unknown fever with hepatosplenomegaly, lymphadenopathy, skin rash, edema and twitch. （3） Laboratory date： all patients had abnormal complete blood counts, coagulopathy with hypoflbrinogemia, hypertriglyceridemia, and hemophagocytosis in bone marrow. 22 cases had hepatic dysfunction and 19 cases had immune dysfunction. （4）Treatment and prognosis： Of total 26 cases, 16 of them were died （61.5%）. The mortality was 47.4% （9/19 cases） in chemotherapy and 100% （7/7 cases） in non-chemotherapy, which showed significant difference （P =0. 023）. The death rate was related to the organ damage over 4 sites （ P = 0. 009）. In 10 survival cases, at least 9 showed EBV activity. Conclusion EBV-HLH is a very serious syndrome with poor prognosis, especially in multiple organ damage patients. The early diagnosis, prompt and proper chemotherapy can improve the survival rate. However, some of patients will continue in the status of CAEBV infection, and may develop into EBV-related lymphoma.