种痘水疱病样淋巴组织增殖性疾病的研究新进展  

Advances in Hydroa Vacciniforme-like Lymphoproliferative Disorder

作　　者：黄亚娇 罗晓燕[1] HUANG Yajiao;LUO Xiaoyan(Department of Dermatology,Children's Hospital of Chongqing Medical University,National Clinical Research Center for Child Health and Disorders,Ministry of Education Key Laboratory of Child Development and Disorders,Chongqing Key Laboratory of Pediatrics,Chongqing 400014,China)

机构地区：[1]重庆医科大学附属儿童医院皮肤科,国家儿童健康与疾病临床医学研究中心,儿童发育疾病研究教育部重点实验室儿科学重庆市重点实验室,儿童感染免疫重庆市重点实验室,重庆400014

出　　处：《皮肤科学通报》2022年第2期90-95,M0002,共7页Dermatology Bulletin

摘　　要：种痘水疱病样淋巴组织增殖性疾病(hydroa vacciniforme-like lymphoproliferative disorder,HVLPD)是一类罕见的与Epstein-Barr病毒(EBV)感染相关的T细胞或NK细胞克隆增殖性疾病,主要影响儿童。既往认为HVLPD是预后良好的光敏性皮肤病。然而,新近研究发现经数月至十余年反复发作后,该病可进展为其他EBV相关T/NK细胞增殖性疾病(EBV^(+)T/NK-LPD),中国患者病死率高达20.0%~47.4%。由于HVLPD的发病机制不清,难以早期预测恶性进展,治疗上尚无异基因造血干细胞移植以外的其他根治措施,为该病管理带来巨大的挑战。本文围绕HVLPD发病机制、治疗及预后相关进展做简要综述。Hydroa vacciniforme-like lymphoproliferative disorder(HVLPD)is a rare Epstein-Barr virus(EBV)-associated T-cell or natural killer(NK)-cell lymphoproliferative disease,which occurs mainly in children.HVLPD was thought to be a photosensitive skin disease with good prognosis.However,recent research has showed that the disease can develop into other EBV-associated T/NK cell lymphoproliferative disease(EBV^(+)T/NK-LPD)after recurrent episodes of several months to more than a decade,with a high mortality of 20%-47.4%in Chinese patients.As the pathogenesis of HVLPD is unclear,it is difficult to predict the malignant progress in the early stage,and there are no radical therapies other than allogeneic hematopoietic stem cell transplantation,which brings great challenges to the management of the disease.This review summarizes the progress of the pathogenesis,therapy and prognosis of HVLPD.

关 键 词：种痘水疱病样淋巴组织增殖性疾病 慢性活动性EB病毒感染 造血干细胞移植 预后 

分 类 号：R725.5[医药卫生—儿科]