首页|EB病毒相关性噬血细胞综合征的治疗进展

EB病毒相关性噬血细胞综合征的治疗进展

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噬血细胞性淋巴组织细胞增生症(HLH)是因免疫应答失常、T淋巴细胞和单核巨噬细胞异常增殖活化引起的严重炎症反应综合征,病情进展快,病死率高。HLH-1994和HLH-2004方案虽然显著提高了病人的生存率,但也存在治疗瓶颈。EB病毒相关性HLH(EBV-HLH)是亚洲儿童最常见的HLH亚型,总体治疗效果好,但仍有20%~30%的患儿复发难治。现就EBV-HLH治疗进展进行综述总结。
Progress in the treatment of Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
Hemophagocytic lymphohistiocytosis(HLH)is a severe inflammatory syndrome caused by the exces-sive proliferation and activation of T cells and macrophages.This disease progresses rapidly with a high mortality rate.Although the HLH-1994 and HLH-2004 protocols have significantly improved patient survival rates,there are still therapeutic challenges.Epstein-Barr virus-associated HLH(EBV-HLH)is the most common subtype of HLH in Asian children.Although the outcome of pediatric EBV-HLH is superior to other subtypes,about 20%~30%of patients experience relapse or arerefractory to the treatment.This review provides an overview of the ad-vancements in the treatment for EBV-HLH.

epstein-barr virushemophagocytic syndrometreatmentprognosis

邢源、陈艳、徐晓军

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遵义医科大学附属医院小儿内科,贵州遵义 563003

浙江大学附属儿童医院肿瘤血液内科,浙江杭州 310003

EB病毒 噬血细胞综合征 治疗 预后

国家自然科学基金面上项目

81970122

2024

遵义医科大学学报
遵义医科大学

遵义医科大学学报

CSTPCD
ISSN:2096-8159
年,卷(期):2024.47(4)
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