Clinical features and prognosis of central nervous system involvement in patients with Epstein -Barr virus associated hemophagocytic lymphohistiocytosis
10.3760/cma.j.issn.2095-428X.2018.06.014
- VernacularTitle:EB病毒相关噬血细胞淋巴组织细胞增生症中枢神经系统受累的临床特点及预后分析
- Author:
Fengyun WEN
1
;
Li XIAO
;
Meiling LIAO
;
Ying XIAN
;
Xianhao WEN
;
Jianwen XIAO
;
Xianmin GUAN
;
Jie YU
Author Information
1. 400014,重庆医科大学附属儿童医院血液与肿瘤中心
- Keywords:
Central nervous system;
Epstein-Barr virus;
Hemophagocytic lymphohistiocytosis
- From:
Chinese Journal of Applied Clinical Pediatrics
2018;33(6):453-457
- CountryChina
- Language:Chinese
-
Abstract:
Objective To investigate the clinical features and prognosis of central nervous system(CNS)in-volvement in Epstein-Barr virus(EBV)associated hemophagocytic lymphohistiocytosis(HLH).Methods A total of 89 patients with EBV-HLH diagnosed in Children's Hospital of Chongqing Medical University from June 2006 to Octo-ber 2015 were divided into involved CNS group and non-involved group according to whether there was CNS involve-ment. The clinical manifestations,laboratory examinations and outcomes of these two groups were analyzed. Results Among these 89 patients with EBV-HLH,39 patients developed CNS disease,19 cases of them had neuro-logical symptoms or signs,including convulsions,unconsciousness,facial palsy,dysarthria,dysphagia,irritability,neck stiffness,Babinski sign positive,opisthotonus;9 cases of them had abnormal cerebrospinal fluid(CSF),with elevated white blood cell count and protein level;26 patients had abnormal brain images,including deepen or widening cortical sulci,atrophy,hemorrhage,high T2 signal in magnetic resonance imaging(MRI).The 3-year survival rate in involved CNS group was lower than those of non-involved group(66.7% vs.86.0%),and there was statistical significance (χ2=4.267,P=0.039).The involved CNS group had higher ferritin(χ2=5.092,3.921;P=0.024,0.048)and lower platelets(Z= -2.643,P=0.008)than those of non-involved group,and there were statistical significances.COX mul-tivariate analysis showed that neurological symptom and abnormal CSF were independent prognostic factors(RR=3.134, 3.339,all P<0.05).Conclusion CNS involvement is frequent in EBV-HLH.The prognosis of children with involved CNS group is worse than those of non-involved group.Neurological symptoms and abnormal CSF are related to poor prognosis.
