80例伴有EB病毒血症的噬血细胞性淋巴组织细胞增生症患者的临床分析

[Clinical analyses of 80 hemophagocytic lymphohistiocytosis patients with Epstein-Barr viremia].

作者信息

Zeng Xiangzong, Wei Na, Wang Yini, Wang Jingshi, Zhang Jia, Wu Lin, Li Shuo, Tang Ran, Huang Wenqiu, Chen Jianhang, Pei Ruijun, Wang Zhao

机构信息

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

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

出版信息

Zhonghua Yi Xue Za Zhi. 2014 Sep 30;94(36):2841-3.

PMID:25534103

Abstract

OBJECTIVE

To analyze the clinical characteristics, treatment and prognosis of hemophagocytic lymphohistiocytosis (HLH) patients with Epstein-Barr viremia (EBV).

METHODS

A retrospective study was conducted to analyze the clinical data of 80 HLH patients aged ≥ 14 years with EBV-DNA >1 000 copies/ml in peripheral blood from 2008 to 2013.

RESULTS

There were EBV-HLH (n = 46), HLH-associated lymphoma (n = 30) and primary HLH (n = 4). Among the relevant laboratory parameters, inter-group statistical differences existed only in alanine transaminase (ALT) and aspartate aminotransferase (AST) (P = 0.021, 0.035). The median follow-up period was 2.0 (0.5-20.0) months. And the 1-month, 3-month, 6-month and 12-month overall survival rates were 58.8%, 37.5%, 29.7% and 19.6% respectively.

CONCLUSIONS

HLH patients with EBV have a pernicious clinical course with a poor prognosis. And it makes little sense for distinguishing HLH-associated lymphoma from EBV-HLH through routine laboratory tests.

摘要

目的

分析伴有EB病毒血症（EBV）的噬血细胞性淋巴组织细胞增生症（HLH）患者的临床特征、治疗及预后。

方法

进行一项回顾性研究，分析2008年至2013年80例年龄≥14岁、外周血EBV-DNA>1000拷贝/ml的HLH患者的临床资料。

结果

其中有EBV-HLH（n=46）、HLH相关淋巴瘤（n=30）和原发性HLH（n=4）。在相关实验室参数中，组间统计学差异仅存在于丙氨酸转氨酶（ALT）和天冬氨酸转氨酶（AST）（P=0.021，0.035）。中位随访期为2.0（0.5-20.0）个月。1个月、3个月、6个月和12个月的总生存率分别为58.8%、37.5%、29.7%和19.6%。