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Atypical Neuroimaging Characteristics of Hemophagocytic Lymphohistiocytosis in Infants: A Case Series of Hemorrhagic Brain Lesions in the Deep Grey Matter Publisher Pubmed



Pak N1, 2 ; Selehnia A2 ; Hunfeld MAW3 ; Lequin MH4 ; Neuteboom RF3 ; De Vries ACH5 ; Kroon AA6 ; Dremmen MHG1
Authors
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Authors Affiliations
  1. 1. Department of Radiology & Nuclear Medicine, Erasmus Medical Center—Sophia Children’s Hospital, P.O. Box 2060, Rotterdam, 3000, CB, Netherlands
  2. 2. Department of Radiology, Tehran University of Medical Sciences—Children’s Medical Center, Tehran, Iran
  3. 3. Department of Neurology, Erasmus Medical Center—Sophia Children’s Hospital, Rotterdam, Netherlands
  4. 4. Department of Radiology, University Medical Center Utrecht—Wilhelmina Children’s Hospital, Utrecht, Netherlands
  5. 5. Department of Pediatrics, Erasmus Medical Center—Sophia Children’s Hospital, Rotterdam, Netherlands
  6. 6. Department of Neonatology, Erasmus Medical Center—Sophia Children’s Hospital, Rotterdam, Netherlands

Source: Neuroradiology Published:2021


Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a rare multisystem condition associated with uncontrolled overproduction and infiltration of lymphocytes and histiocytes predominantly in liver, lymph nodes, spleen, and central nervous system. Neuroimaging findings on MRI are fairly nonspecific and classically include periventricular white matter signal abnormalities and diffuse atrophy. Focal parenchymal lesions may demonstrate post contrast ring or nodular enhancement and calcification. However, the MR imaging characteristics can be highly variable. Here, we present two cases of HLH in infants with multiple hemorrhagic lesions mostly depicted in both thalami and basal ganglia regions. Thalamic, basal ganglia, and brain stem involvement with hemorrhagic changes in HLH are rarely described in literature. Early diagnosis of HLH may be lifesaving. Awareness of the disease is necessary to investigate its characteristic findings and avoiding a delay in diagnosis. © 2020, The Author(s).