Background: Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening clinical syndrome. Central nervous system (CNS) involvement is a severe complication, which can lead to rapid disease development and higher morality. However, this has not been given enough attention in adult HLH. Therefore, we carried out this study to analyze the clinical features, laboratory findings, treatment outcomes, and other characteristics of adult HLH with CNS involvement.
Methods: A retrospective analysis of 96 adult patients with HLH combined with CNS involvement between June 2003 and December 2016 was conducted. Clinical features, cerebrospinal fluid (CSF) features, image changes, and therapeutic outcomes were analyzed.
Results: Among the 96 patients, 86 had various CNS symptoms and 33 (38.4%) had already presented symptoms before the HLH diagnosis was confirmed. A total of 59 patients received CSF examinations and showed abnormalities in 23 patients (39.0%). Seventy patients received imaging examinations and the results showed fifty patients with imaging changes (71.4%). Fifty-seven patients received multiple rounds of repeated intrathecal injection therapy and 35 patients improved (61.4%). As for the multiple analyses of effective factors on survival time, the results showed that the effects of combined Epstein-Barr virus (EBV) infection (P = 0.026, Exp(B) = 2.309, 95% confidence interval [CI] [1.108, 4.823) and intrathecal injection therapy (P = 0.013, Exp(B) = 0.422, 95% CI [0.214, 0.831]) on the survival time of the CNS-HLH patients were significant.
Conclusions: Complication with EBV infection is a risk factor, and intrathecal injection is a protective factor. CNS involvement in HLH is not rare, which can result in a poor prognosis. Multiple rounds of repeated intrathecal injection therapy can improve the prognosis of CNS-HLH patients.
成人噬血细胞淋巴组织增多症伴中枢神经系统受累：单中心96例回顾性分析摘要背景：噬血细胞淋巴组织增多症（hemophagocytic lymphohistiocytosis，HLH）是一类威胁生命的临床综合征。中枢神经系统（central nervous system，CNS）受累是其一种严重的并发症，进展较快，死亡率增加，但在成人HLH中未得到广泛重视。因此，我们通过观察这类患者，来分析成人HLH伴CNS受累的临床表现、实验室检查、治疗预后等特点。 方法：回顾性分析2003年6月至2016年12月我中心96例HLH伴CNS受累成人患者的临床资料、脑脊液变化、影像学异常及治疗、转归。 结果：在收录的96名患者中，86名存在神经系统症状，其中33名（38.4%）在HLH确诊前即出现神经系统症状。59名患者接受脑脊液检查，其中23名（39.0%）存在异常。70名患者接受影像学检查，其中50名（71.4%）出现影像学改变。共57名患者接受多次鞘内注射治疗，其中35名（61.4%）患者情况改善。对于影响预后的多因素分析发现，合并EB病毒（Epstein-Barr virus，EBV）感染（P=0.026，Exp(B)=2.309，95% CI [1.108，4.823]）及接受鞘内注射治疗（P=0.013, Exp(B)=0.422，95% CI [0.214,0.831]）对生存时间的影响有统计学意义。 结论：对于成人HLH合并CNS受累的患者，存在EB病毒感染提示不良预后，而接受鞘内注射治疗则可改善预后。成人当中的HLH合并CNS受累并不少见，并且一旦出现CNS受累提示预后较差。多疗程的鞘内注射治疗可以有效改善这类CNS-HLH患者的预后。.
Keywords: Central Nervous System; Hemophagocytic Lymphohistiocytosis; Neuroimaging; Treatment.