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.
作者
黄亚娇
罗晓燕
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)
出处
《皮肤科学通报》
2022年第2期90-95,M0002,共7页
Dermatology Bulletin
作者简介
通信作者:罗晓燕,主任医师/副教授,硕士生导师。重庆医科大学儿童医院皮肤科主任,皮肤教研室主任。美国约翰霍普金斯医学院过敏与临床免疫中心博士后。主要从事儿童炎症性皮肤病免疫机制,,过敏与遗传。中华医学会皮肤性病学分会儿童学组秘书,中华医学会变态反应学分会皮肤学组委员,中华医学会皮肤性病学分会实验学组委员,中国妇幼保健协会儿童变态反应专委会常务委员,重庆市预防医学会变态反应专委会副主任委员,重庆市医学会变态反应分会副主任委员,重庆医师协会皮肤性病学分会遗传罕见病学组组长,重庆市卫健委中青年医学高端人才。主持国家自然科学基金、欧莱雅健康人皮肤基金、省市级基金共8项。发表JCI insight JA-MA Dermatology、Frontiers in Immunology等SCI文章20篇。E-mail:luoxycq@hotmail.com。
