Objective:To investigate the MRI characteristics of brain parenchymal lesions in patients with primary hemophagocytic lymphohistiocytosis(HLH)and enhance the diagnostic accuracy of primary HLH.Materials and Methods:The brain MRI findings and clinical data of 28 patients with primary HLH between December 2016 and August 2023 were retro-spectively analyzed.The MRI analysis encompassed the assessment of location,shape,size,and signal characteristics of brain parenchymal lesions,measurement of ventricular size index.Clinical symptoms,cerebrospinal fluid routine and biochemical were compared and analyzed.Results:Based on lesion morphology and extent,brain parenchymal lesions of primary HLH were divided into 3 types:Punctate lesions and/or periventricular cap lesions in deep white matter(type Ⅰ,15 cases,62.5％).Patchy lesions and/or periventricular halo lesions in deep white matter(type Ⅱ,3 cases,12.5％).Diffuse patchy lesions in deep white matter(type Ⅲ,6 cases,25％),additionally,two cases were complicated with bilateral caudate nucleus lesions and two cases were complicated with multiple intracerebral hemorrhages.The mean ventricular size index of the three type lesions were 32.6％,34％and 34.6％,respectively.Four patients presented with central nervous system symptoms and 3 patients were detected type Ⅲ lesions.Two patients showed increased CSF cell count or protein.Among the 10 patients with elevated CSF cell count or protein,MRI examination revealed no abnormalities in brain parenchyma for two cases,five cases exhibited typeⅠ lesion patterns,and three cases demonstrated type Ⅲ lesion patterns.In contrast,in 10 patients with normal CSF cell count or protein,MRI examination showed type Ⅰ lesions in 6 cases,type Ⅱ lesions in 3 cases,and type Ⅲ lesions in 1 case.Conclusions:Central nervous system involvement is frequently observed in primary HLH.MRI often reveals hyperintensity in the deep white matter or periventricular white matter,accompanied by varying degrees of ventricular dilatation or brain atro-phy,as well as single or multiple hemorrhages.Different types of brain parenchymal lesions can partially reflect the severity of the disease.
维普
万方数据