Central Nervous System Involvement in 179 Chinese Children with Hemophagocytic Lymphohistiocytosis.
Central Nervous System Involvement in 179 Chinese Children with Hemophagocytic Lymphohistiocytosis.
复制标题
179 例中国噬血细胞淋巴组织细胞增多症儿童的中枢神经系统受累情况
DOI：
10.4103/0366-6999.237409
复制
发表时间：
2018-08-05
影响因子：
6.1
通讯作者：
Zhang R
中科院分区：
文献类型：
--
作者：
Zhao YZ;Zhang Q;Li ZG;Zhang L;Lian HY;Ma HH;Wang D;Zhao XX;Wang TY;Zhang R
背景：许多嗜血球性淋巴组织细胞增多症（HLH）患者累及中枢神经系统（CNS）。在本研究中，我们主要分析HLH患儿的神经系统症状、影像学表现、脑脊液（CSF）及其与预后的关系。方法：回顾性分析2010年1月至2015年12月我院收治的179例小儿HLH患者的相关资料。诊断和治疗依据HLH-2004方案。采用双尾卡方检验比较各组间差异，采用Kaplan-Meier生存曲线分析HLH患者总生存期（OS）。结果：在本研究中，21.2%（38/179）的患者有神经系统症状，包括癫痫发作、烦躁、嗜睡和意识不清。颅内影像学异常80例（50.0%，排除无影像学资料19例）。脑脊液结果异常的患者占14.7%（17/116，不包括63例未行腰椎穿刺的患者）。中枢神经系统受累定义为中枢神经系统症状、影像学表现和脑脊液的一种或多种异常。因此，60.3%的患者有中枢神经系统受累。预后方面，中位随访时间为3.2年（失访17年）。无中枢神经系统受累患儿的3年OS（86.0%±4.6%）高于有中枢神经系统受累患儿（68.9%±4.9%，风险比[HR] = 2.286, P = 0.019）。其中，无中枢神经系统症状患儿的3年OS为76.0%±3.8%，高于有中枢神经系统症状患儿（59.5%±8.1%,HR = 2.147, P = 0.047）。脑脊液异常患儿3年OS为64.7%±11.6%，脑脊液正常患儿为85.1%±3.7%,HR = 0.255, P = 0.038。结论：伴有中枢神经系统受累的HLH患者预后较不伴有中枢神经系统受累的患者差，中枢神经系统症状和脑脊液改变对预后的影响比影像学异常更重要。
Background: Central nervous system (CNS) involvement is found in many patients with hemophagocytic lymphohistiocytosis (HLH). In this study, we mainly analyzed neurological symptoms, imaging findings, cerebrospinal fluid (CSF), and their relationship with outcomes of HLH children. Methods: Related data of 179 Chinese pediatric patients with HLH admitted to our center from January 2010 to December 2015 were analyzed retrospectively. Diagnosis and treatment were based on the HLH-2004 protocol. Two-tailed Chi-squared test was used to compare between different groups, and Kaplan-Meier survival curves were used to analyze the overall survival (OS) of patients with HLH. Results: In the present study, 21.2% (38/179) of total patients had neurological symptoms including seizure, irritability, somnolence, and unconsciousness. There were 80 (50.0%, excluding 19 patients without imaging data) patients with cranial imaging abnormalities. There were 14.7% (17/116, excluding 63 patients who did not accept lumbar puncture) of patients with abnormal CSF results. CNS involvement is defined as abnormalities in one or more of CNS symptoms, radiological findings, and CSF. Thus, 60.3% of them had CNS involvement. As for the prognosis, the median follow-up time was 3.2 years (17 lost to follow-up). The probable 3-year OS of children was higher without CNS involvement (86.0% ± 4.6%) than those with CNS involvement (68.9% ± 4.9%, hazard ratio [HR] = 2.286, P = 0.019). Among them, the probable 3-year OS of children without CNS symptoms was 76.0% ± 3.8%, higher than with CNS symptoms (59.5% ± 8.1%, HR = 2.147, P = 0.047). The 3-year OS of children with abnormal CSF was 64.7% ± 11.6%, compared with normal CSF (85.1% ± 3.7%, HR = 0.255, P = 0.038). Conclusions: HLH patients with CNS involvement might have worse outcomes compared with those without CNS involvement, and CNS symptoms and CSF changes are more important to access the prognosis than imaging abnormality.
登录
查看更多内容
DOI：
10.5045/kjh.2012.47.4.273
发表时间：
2012-12
期刊：
The Korean journal of hematology
影响因子：
--
作者：
Kim MM;Yum MS;Choi HW;Ko TS;Im HJ;Seo JJ;Koh KN
通讯作者：
Koh KN
影响因子：
2.1
作者：
Anderson, Tara L.;Carr, Carrie M.;Kaufmann, Timothy J.
通讯作者：
Kaufmann, Timothy J.
影响因子：
6.5
作者：
HIRST, WJR;LAYTON, DM;PRITCHARD, J
通讯作者：
PRITCHARD, J
影响因子：
3.2
作者：
Yang, Shuang;Zhang, Li;Shen, Kunling
通讯作者：
Shen, Kunling
影响因子：
3.1
作者：
Koh, Kyung-Nam;Im, Ho Joon;Seo, Jong Jin
通讯作者：
Seo, Jong Jin