上海交通大学学报（医学版）

上海交通大学学报（医学版）

• 论著（临床研究） • 上一篇    下一篇

成人嗜血细胞性淋巴组织增生症伴中枢神经系统受累系统分析

高枚春，陆钦池   

  1. 上海交通大学 医学院附属仁济医院神经内科， 上海 200127
  • 出版日期:2015-11-28 发布日期:2016-01-13
  • 通讯作者: 陆钦池, 电子信箱: qinchilu@yahoo.com。
  • 作者简介:高枚春（1983—）， 女， 住院医师， 硕士; 电子信箱: crayonxc@hotmail.com。

Systematic analysis of adults with hemophagocytic lymphohistiocytosis and combined with central nervous system #br# involvement

GAO Mei-chun, LU Qin-chi   

  1. Department of Neurology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai 200127, China
  • Online:2015-11-28 Published:2016-01-13

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摘要：

目的  回顾性分析文献报道，总结成人嗜血细胞性淋巴组织增生症（HLH）伴中枢神经系统(CNS)受累的临床特征。方法  通过检索万方、维普、中国知网（CNKI）、PubMed和Ovid搜索引擎，对1950年1月—2014年12月发表的有关成人HLH伴CNS受累的报道进行系统分析。结果  共收集21例伴有CNS受累的成人HLH患者，其临床表现各异，以意识改变(66.7%)、癫痫(33.3%)、构音障碍(19.0%)和共济失调(19.0%)最为常见。影像学改变并非特异，最常累及脑深部白质（53.3%），其次为脑干（40.0%）和小脑（26.7%），部分病例增强扫描可见强化。脑脊液特征多为轻至中度的细胞数增多和不同程度的蛋白含量增高，葡萄糖水平正常。继发于自身免疫病的HLH更易累及CNS（38.1%）。总死亡率为81.0%。结论  成人HLH累及CNS者死亡率高，预后极差，早期诊治、避免CNS浸润是关键。

关键词: 成人, 嗜血细胞性淋巴组织增生症, 嗜血细胞综合征, 中枢神经系统, 临床特征, 系统分析

Abstract:

Objective  To retrospectively analyze the literature and summary the clinical features of adults with  hemophagocytic lymphohistiocytosis (HLH) and combined with central nervous system (CNS) involvement. Methods  Reports of adults with HLH and combined with CNS involvement from January 1950 to December 2014 were retrieved from databases of Wanfang, VIP, China National Knowledge Infrastructure (CNKI), PubMed, and Ovid and systematically analyzed. Results  A total of 21 cases of adults with HLH and combined with CNS involvement were collected. Their clinical manifestations varied and the most common ones were altered consciousness (66.7%), epilepsy (33.3%), dysarthria (19.0%), and ataxia (19.0%). The image changes were not specific and the most frequently involved areas were deep brain white matter (53.3%), followed by brain stem (40.0%) and cerebellum (26.7%) and some cases showed enhancement of lesions by enhanced scan. The features of cerebrospinal fluid were mild to moderate pleocytosis, different degrees of elevated protein content, and normal glucose level. The HLH secondary to autoimmune diseases was more likely to involve the CNS (38.1%) and the total mortality was 81.0%. Conclusion  Adults with HLH and involved CNS have high mortality and poor prognosis. Early diagnosis and treatment and prevention of CNS infiltration are key factors.

Key words: adult, hemophagocytic lymphohistiocytosis, hemophagocytic syndrome, central nervous system, clinical features, systems analysis