EBV驱动相关噬血细胞性淋巴组织细胞增多症

doi:10.3969/j.issn.1004-583X.2021.10.009

临床荟萃 ›› 2021, Vol. 36 ›› Issue (10): 910-915.doi: 10.3969/j.issn.1004-583X.2021.10.009

• 专题:血液病诊疗关键技术转化与推广 • 上一篇下一篇

EBV驱动相关噬血细胞性淋巴组织细胞增多症

崔亭亭, 张嘉, 王晶石, 王昭()

首都医科大学附属北京友谊医院血液内科, 北京 100050

收稿日期:2021-10-05 出版日期:2021-10-20 发布日期:2021-11-10
通讯作者: 王昭 E-mail:wangzhao@ccmu.edu.cn
作者简介:王昭,二级主任医师/教授,博士生(后)导师,首都医科大学附属北京友谊医院血液内科主任。国际组织细胞协会噬血细胞综合征执行委员,中国医师协会血液科医师分会组织细胞疾病专业委员会主任委员,中华医学会血液学分会淋巴细胞疾病学组副组长,中国免疫学会血液免疫分会常委,中国抗癌协会淋巴瘤专业委员会常委,中国抗癌协会血液病转化医学专业委员会常委,中国老年学学会老年肿瘤专业委员会淋巴血液肿瘤分委会常委,中国妇幼保健协会脐带血临床应用专业委员会常委,海峡两岸医药卫生交流协会血液病专家委员会常委、总干事长。北京市医管局扬帆计划重点专业团队负责人、登峰人才团队负责人。主持噬血细胞综合征中国专家共识的制定并参与噬血细胞综合征国际指南的制定。第一完成人获得中华医学科技奖二等奖、华夏医学科技二等奖、北京医学科技二等奖、“金桥奖”二等奖、北京市科学技术三等奖。主编《噬血细胞综合征》《组织细胞疾病》专著,担任《中华血液学杂志》《中华医学杂志》《中国实验血液学杂志》《白血病·淋巴瘤》《临床内科杂志》等编委,Haematologica, British Journal of Haematology,Orphanet Journal of Rare Diseases等国际期刊审稿人。

Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis

Cui Tingting, Zhang Jia, Wang Jingshi, Wang Zhao()

Department of Hematology, Beijing Friendship Hospital, Capital Medical University, Beijing 100050, China

Received:2021-10-05 Online:2021-10-20 Published:2021-11-10
Contact: Wang Zhao E-mail:wangzhao@ccmu.edu.cn

摘要/Abstract

摘要：

噬血细胞性淋巴组织细胞增多症(hemophagocytic lymphohistiocytosis, HLH)是由于机体免疫功能失控,形成HLH致命的炎症因子风暴表现。随着分子遗传学研究的发展,目前,对于HLH的认识逐渐深入。其中,一些基因突变在引起HLH过程中,EBV感染为其重要诱因,这类疾病被称为EB病毒驱动型噬血细胞性淋巴组织细胞增多症。目前已知基因包括IL-2诱导的T细胞激酶缺乏(ITK)缺陷、镁离子转运体1(MAGT1)缺陷、CD27缺陷、CD70缺陷、CTPS1缺陷以及RASGRP1缺陷。本文将对EB病毒驱动型噬血细胞性淋巴组织细胞增多症的流行病学、病理生理机制、临床表现、治疗进行综述。

关键词: 淋巴组织细胞增多症,噬血细胞性, 疱疹病毒4型,人

Abstract:

Hemophagocytic lymphohistiocytosis (HLH) is the life-threatening result of inflammatory storm caused by immunological dysfunction. With the development of molecular genetics, current studies have an in-depth understanding of the HLH. For some genotype of HLH, epstein-barr virus (EBV) is an important inducement, known as the EBV-driven HLH. Currently, the Tyrosine-protein kinase ITK deficiency, magnesium transporter protein 1 (MAGT1) deficiency, CD27 deficiency, CD70 deficiency, CTP synthase 1 (CTPS1) deficiency and RAS guanyl-releasing protein 1 (RASGRP1) deficiency are found to be the genotype related to the HLH. In this review, we would summarize the epidemiology, pathophysiological characteristics, clinical manifestations and treatment of EBV-driven HLH.

Key words: lymphohistiocytosis,hemophagocytic, herpesvirus 4,human

中图分类号:

R551.12

崔亭亭, 张嘉, 王晶石, 王昭. EBV驱动相关噬血细胞性淋巴组织细胞增多症[J]. 临床荟萃, 2021, 36(10): 910-915.

Cui Tingting, Zhang Jia, Wang Jingshi, Wang Zhao. Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis[J]. Clinical Focus, 2021, 36(10): 910-915.

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链接本文: https://huicui.hebmu.edu.cn/CN/10.3969/j.issn.1004-583X.2021.10.009

https://huicui.hebmu.edu.cn/CN/Y2021/V36/I10/910

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EBV驱动相关噬血细胞性淋巴组织细胞增多症

Epstein-Barr virus-driven hemophagocytic lymphohistiocytosis

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