Learning objectives
This educational poster will highlight the imaging findings in our cohort of paediatric patients with confirmed HLH.
The reader will:
Understand the wide range of imaging findings associated with HLH.
Appreciate the radiological mimics of intracranial HLH.
Apply their knowledge when protocolling studies for suspected HLH to include the relevant sequences.
Background
Haemophagocytic Lymphohistiocytosis (HLH) is a rare proliferative disorderthat can result in lymphocytic infiltration of multiple organs including the central nervous system (CNS). CNS involvement can present with seizures, confusion, reduced GCS and ataxia.
The neuroradiological findings in HLH are varied and can often mimic other pathologies including infarction, metabolic conditions and rarely child abuse [1]. It is therefore important for any radiologist reporting brain MRI to appreciate the variety of presentations that exist and be able to include HLH in the differential if appropriate.
Findings and procedure details
Leeds Children’s Hospital is a tertiary centre for paediatric haematology and oncology with the capability of offering stem cell transplantation. This means it is one of the largest referral bases in the UK. Since 2006, 15 of the patients with suspected intracranial HLH have been studied with MRI with 12/15 confirming intracranial manifestations of HLH.
We have performed a retrospective review of the imaging to illustrate the variety of MRI findings present in this cohort. This includes; deep white matter hyperintensities, cortical diffusion changes, parenchymal...
Conclusion
CNS HLH may be suspected clinically but the radiological findings often mimic other conditions that can present in similar ways. Radiologists reporting brain MRI should be aware of rare diseases which can mimic more common pathologies to enable them to provide a thorough differential.
Personal information and conflict of interest
S. Hussain; Leeds/UK - nothing to disclose B. James; Leeds/UK - nothing to disclose P. J. Shah; Leeds/UK - nothing to disclose K. E. Twentyman; Leeds/UK - nothing to disclose S. Currie; Leeds/UK - nothing to disclose I. Craven; Leeds/UK - nothing to disclose
References
1.Pediatrics.2003 May;111(5 Pt 1): e636-40