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

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.