Prognosis of the central nervous system involvement in patients with hemophagocytic lymphohistiocytosis
10.3760/cma.j.issn.0253-2727.2017.10.005
- VernacularTitle: 中枢神经系统受累的噬血细胞淋巴组织细胞增生症预后分析
- Author:
Fengyun WEN
1
;
Li XIAO
;
Ying XIAN
;
Xianhao WEN
;
Xianmin GUAN
;
Meiling LIAO
;
Jie YU
Author Information
1. Department of Hematology and Oncology, Children’s Hospital of Chongqing Medical University, Chongqing 400014, China
- Publication Type:Journal Article
- Keywords:
Lymphohistiocytosis, hemophagocytic;
Central nerous system;
Prognosis
- From:
Chinese Journal of Hematology
2017;38(10):848-852
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the characteristics and prognostic factor of central nervous system (CNS) involvement in patients with hemophagocytic lymphohistiocytosis (HLH) .
Methods:From January 2006 to October 2015, 152 patients with HLH, 88 patients had CNS involvement, their clinical data were collected, and survival was analyzed using the Kaplan-Meier life table method, univariate and multivariate Cox regression model analyses were applied to identify the risk factors of prognosis.
Results:①57.9% patients complicated with neurological symptoms, cerebrospinal fluid abnormalities were observed in 37.0% patients, 57.5% patients had abnormal neuroradiology. ②36 patients survived well, 3 patients lost to follow-up, 49 dead, 1 survival patient had epilepsy. ③The 3-year overall survival rate of 88 patients was 44%. ④abnormal CSF and unreceived IT bore a significant, independent adverse prognostic value (P<0.05) .
Conclusion:CNS involvement in HLH has a high frequency and poor prognosis, few patients remained neurologic sequelae; abnormal CSF related to poor prognosis, positive intrathecal injections could improve the prognosis.
