Mri Finding And Clinical Of Correlation Central Nervous System Involvement In Children With Haemophagocytic Lymphohistiocytosis

Objective:To study MRI features and their clinical significance of central nervous system(CNS)involvement in children with haemophagocytic lymphohistiocytosis(HLH).Materials:Imaging and clinical data of 118 children with CNS-HLH referred to our institution from January 2012 to June 2019 were retrospectively analyzed.The patients were then grouped upon neurological imaging features.We utilized non-parametric test(Kruskal-Wallis rank sum test and Spearman correlation coefficient)to analyze the differences and correlations of the clinical independent risk factors in each group.The clinical independent risk factors including cerebrospinal fluid(CSF)microprotein content,CSF cell counts,aspartate aminotransferase(AST),lactate dehydrogenase(LDH),fibrinogen(FIB),platelets(PLT)and activated partial thromboplastin time(APTT).Results:Patients were divided into three groups,including normal appearance(Group 1,17/118,14.4%),encephalatrophy-like appearance(Group 2,44/118,37.2%),and brain parenchyma lesions(Group 3,57/118,48.3%).Among them,the imaging finding of children in Group 3were further divided into three subtypes: small lesions(45.7%),patchy or diffuse lesions(42.9%)and haematomas(5.2%).70.2% children in the third group were accompanied by "brain-atrophy" changes.Comparing biochemical values among the three groups revealed a significant difference for all values(p < 0.05),except for cell counts of CSF.Pairwise comparisons were performed,P-value corrected by the Bonferroni method,revealing significant differences in AST(p = 0.012)and LDH(p = 0.009)between Group 1 and Group 2;in CSF protein content(p < 0.001),AST(p < 0.001),LDH(p < 0.001),FIB(p = 0.007),APTT(p = 0.001)and PLT(p < 0.001)between Group 1 and 3;and in CSF microprotein content(p = 0.022),AST(p = 0.002),LDH(p = 0.003)and PLT(p < 0.001)between Group 2 and 3.Spearman's rank correlation coefficient also demonstrated that CSF cell counts(r = 0.193,p = 0.036),CSF protein content(r = 0.379,p < 0.001),AST(r = 0.521,p < 0.001),LDH(r = 0.514,p < 0.001)and APTT(r = 0.326,p < 0.001)were positively correlated with the image group ranks,while PLT(r =-0.633,p < 0.001)and FIB(r =-0.258,p = 0.005)were negatively correlated with them.Conclusion : CNS-HLH MRI finding is diverse and nonspecific,among which the predominant feature is encephalatrophy-like appearance,followed by brain parenchymal lesions.The imaging grouping has a certain correlation with the clinical independent risk factors,which reflects the severity of the disease and can be used as important visible evidence for the clinical diagnosis of brain involvement in HLH and its complications.