儿童噬血细胞性淋巴组织细胞增生症中枢神经系统受累的影像特点及临床相关性

Imaging characteristics and clinical relevance of central nervous system involvement in children with hemophagocytic lymphohistiocytosis

摘要:

目的：研究儿童噬血细胞性淋巴组织细胞增生症（hemophagocytic lymphohistiocytosis，HLH）中枢神经系统（central ner－vous system，CNS）受累的影像表现及其与临床的相关性。方法：回顾性分析2012年1月至2018年11月于重庆医科大学附属儿童医院确诊的90例HLH中枢神经系统受累（CNS-HLH）患儿的影像和临床资料，按其影像表现分为3组，即阴性组（第1组）、蛛网膜下腔和（或）脑室扩大组（第2组）、脑实质病变组（第3组）。利用趋势性卡方检验分析各组间脑脊液检查阳性率、脑电图阳性率和临床表现阳性率的变化趋势，非参数检验（Kruskal-Wallis秩和检验）分析各组患儿独立风险因素的差异性。结果：第1组30例（33.3%），第2组34例（37.8%），第3组26例（28.9%）；CNS-HLH头颅影像表现多样，其中，第3组患儿影像学表现可分为小病灶（46.2%）、大片或弥漫性病灶（34.6%）、出血性病灶（19.2%）３型。3组患儿的脑脊液检查阳性率、脑电图阳性率、临床表现阳性率与组序号呈线性正相关（均P＜0.05）。3组患儿的脑脊液（cerebrospinal fluid，CSF）微量蛋白含量及细胞数、血小板计数（platelet count，PLT）具有差异性（均P＜0.05），组间两两比较，P值经Bonferroni校正显示，第1组与第2组之间仅有CSF细胞数有统计学差异（均P＜0.05），第1组与第3组及第2组与第3组间的CSF蛋白含量、细胞数和PLT均有统计学差异（均P＜0.05）。结论：HLH脑部影像具有一定的规律性，可反映疾病的严重程度和预后，为临床调整治疗方案提供影像学依据。

Abstract:

Objective：To study the imaging characteristics and clinical relevance of central nervous system（CNS） involvement in chil－dren with hemophagocytic lymphohistiocytosis（HLH）. Methods：The imaging and clinical data of 90 children diagnosed with CNS-HLH in our hospital from January 2012 to November 2018 were retrospectively analyzed. According to the imaging characteristics，the children were divided into three groups，namely normal group（Group 1），subarachnoid space and/or ventricular enlargement group （Group 2），and brain parenchymal lesions group（Group 3）. The chi-square test for trend was used to analyze the change tendency of the positive rates of cerebrospinal fluid（CSF） test，electroencephalogram（EEG），and clinical manifestations，and the non-parametric test（Kruskal-Wallis test） was used to analyze the intergroup differences in independent risk factors. Results：There were 30 children in Group 1（33.3%），34 children in Group 2（37.8%），and 26 children in Group 3（28.9%），with different brain images between the three groups. The imaging findings of Group 3 could be divided into small lesion type（46.2%），large or diffuse lesion type（34.6%），and hemorrhagic lesion type（19.2%）. The positive rates of CSF test，EEG，and clinical manifestations in the three groups were positively correlated with their group numbers（P<0.05 for all）. The microprotein content，cell number，and platelet count（PLT） of CSF were significantly different between the three groups（P<0.05 for all）. After pairwise comparison and Bonferroni correction of P value，Group 1 and Group 2 were only significantly different in CSF cell count（P<0.05），while there were significant differences in the microprotein content，cell number，and PLT of CSF between Group 1 and Group 3 and between Group 2 and Group 3（P<0.05 for all）. Conclusion：The brain images of HLH can reflect the severity and prognosis of the disease and provide an imaging basis for the adjustment of clinical treatment.