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  

Clinical features and prognosis of central nervous system involvement in patients with Epstein - Barr virus associated hemophagocytic lymphohistiocytosis

作　　者：文凤云 肖莉[1] 廖美玲 宪莹[1] 温贤浩[1] 肖剑文[1] 管贤敏[1] 于洁[1] Wen Fengyun;Xiao Li;Liao Meiling;Xian Ying;Wen Xianhao;Xiao Jianwen;Guan Xianmin;Yu Jie(Department of Hematology and Oncology, Children＇s Hospital of Choagqing Medical University, Chongqing 400014, China)

机构地区：[1]重庆医科大学附属儿童医院血液与肿瘤中心,400014

出　　处：《中华实用儿科临床杂志》2018年第6期453-457,共5页Chinese Journal of Applied Clinical Pediatrics

摘　　要：目的了解EB病毒（EBV）相关噬血细胞淋巴组织细胞增生症（HLH）中枢神经系统（CNS）受累的临床特点及预后。方法2006年6月至2015年10月在重庆医科大学附属儿童医院诊治的89例EBV－HLH患儿，根据有无CNS受累分为CNS受累组和非CNS受累组，回顾性分析2组的临床表现、初诊时的实验室检查结果及治疗转归。结果89例EBV－HLH患儿中，39例出现CNS受累；19例患儿有神经症状体征，包括抽搐、意识改变、面神经麻痹、构音障碍，吞咽困难、激惹、巴氏征阳性、颈强直、角弓反张；9例患儿脑脊液白细胞数增多或蛋白升高；26例患儿头颅影像学异常，包括脑沟加深增宽、脑萎缩、出血、MRI上T2高信号等。CNS受累组患儿较非CNS受累组患儿3年生存率低（66.7%比86.0%），差异有统计学意义（χ2＝4.267，P＝0.039）。CNS受累组患儿铁蛋白增高程度显著高于非CNS受累组，差异有统计学意义（χ2＝5.092、3.921，P＝0.024、0.048）；血小板水平显著低于非CNS受累组，差异有统计学意义（Z＝－2.643，P＝0.008）。COX多因素分析提示神经系统症状、脑脊液异常是预后的独立危险因素（RR＝3.134、3.339，均P〈0.05）。结论CNS受累在EBV－HLH较为常见。CNS受累的患儿预后较非CNS受累患儿差。神经症状、脑脊液异常与预后关系密切。ObjectiveTo investigate the clinical features and prognosis of central nervous system （CNS） involvement in Epstein-Barr virus（EBV） associated hemophagocytic lymphohistiocytosis （HLH）.MethodsA total of 89 patients with EBV-HLH diagnosed in Children′s Hospital of Chongqing Medical University from June 2006 to October 2015 were divided into involved CNS group and non-involved group according to whether there was CNS involvement.The clinical manifestations, laboratory examinations and outcomes of these two groups were analyzed.ResultsAmong these 89 patients with EBV-HLH, 39 patients developed CNS disease, 19 cases of them had neurological 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）.ConclusionCNS 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.

关 键 词：中枢神经系统 EB病毒 噬血细胞淋巴组织细胞增生症 

分 类 号：R725.5[医药卫生—儿科]