Abnormalities on brain MR images during the course of familial haemophagocytic lymphohistocytosis in a child. A case report

Research output: Contribution to journalArticle


PURPOSE: To describe and report the neuroradiological findings and clinical features in a patient with familial haemophagocytic lymphohistocytosis (FHL), a rare hereditary immune dysregulation in early childhood characterised by multisystem involvement, including in approximately 30% of cases also the central nervous system (CNS).

MATERIAL AND METHODS: Serial brain MR examinations were carried out in a 4.5-year-old boy with FHL, finally complicated with Epstein-Barr virus (EBV)-driven posttransplantation lymphoma.

RESULTS: Multiple brain MR examinations before and after contrast enhancement showed discrete perivascular non-enhancing areas of high signal intensity on T2 images, and later also an enhancing lesion in the right caudate nucleus.

CONCLUSION: FHL should be included in the differential diagnosis of patchy white matter abnormalities in young patients. EBV-driven post-transplantation lymphoma, which may present as meningial and/or parenchymal CNS infiltration, is a differential diagnostic problem.


External organisations
  • Skåne University Hospital
Research areas and keywords


  • Brain, Child, Preschool, Diagnosis, Differential, Histiocytosis, Non-Langerhans-Cell, Humans, Infant, Infant, Newborn, Magnetic Resonance Imaging, Male
Original languageEnglish
Pages (from-to)22-6
Number of pages5
JournalActa Radiologica
Issue number1
Publication statusPublished - 2000 Jan
Publication categoryResearch