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Radiological findings of Posterior Reversible Encephalopathy Syndrome in transplanted children previous affected by

Eliseo Picchi1, Francesca Di Giuliano1, Simone Marziali1

  • 1Diagnostic Imaging and Neuroradiology, Department of Biomedicine and Prevention, University of Rome Tor Vergata, Via Montpellier 1, 00133 Rome, Italy.

European Journal of Radiology Open
|April 25, 2019
PubMed

Insights

Posterior reversible encephalopathy syndrome (PRES) in children undergoing hematopoietic stem cell transplant for hemoglobinopathies is typically mild. Magnetic Resonance Imaging (MRI) showed no significant difference in PRES pattern or severity between thalassemia and sickle cell disease patients.

Area of Science:

  • Pediatric Neurology
  • Neuroradiology
  • Hematopoietic Stem Cell Transplantation

Background:

  • Posterior Reversible Encephalopathy Syndrome (PRES) can occur post-transplant, particularly in children with hemoglobinopathies like thalassemia (THAL) and sickle cell disease (SCD).
  • Understanding the typical presentation and severity of PRES in this population is crucial for patient management.
  • Autologous hematopoietic stem cell transplantation (aHSCT) is a treatment option for these conditions.

Purpose of the Study:

  • To determine the typical pattern and severity of PRES in pediatric patients with hemoglobinopathies undergoing transplantation.
  • To compare PRES patterns and severity between thalassemia and sickle cell disease patients post-aHSCT.
  • To identify any atypical PRES presentations or specific central nervous system involvement.

Main Methods:

  • Retrospective analysis of brain MRI scans from 21 pediatric patients with THAL or SCD who developed PRES after aHSCT and presented with neurological symptoms.
  • Neuroradiologists assessed PRES patterns using the Bartynski and Boardman classification and severity using the McKinney scale.
  • Statistical analysis (Fisher Exact Probability test or Chi-square test) was used to compare categorical data.

Main Results:

  • PRES severity was predominantly mild in 85.7% of the transplanted children.
  • No preferred radiological pattern for PRES was identified on MRI.
  • No significant association was found between PRES pattern or severity and the type of hemoglobinopathy (THAL vs. SCD).

Conclusions:

  • PRES in children with hemoglobinopathies undergoing transplantation is typically mild and does not show a specific radiological pattern.
  • Sickle cell disease patients, despite potential endothelial damage, did not exhibit different PRES severity or patterns compared to thalassemia patients post-aHSCT.
  • No atypical PRES presentations were observed in this cohort.

Keywords:
HemoglobinopathyMRIPRESSickle-cell diseaseThalassemiaaHSCT

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