Therapeutic Plasma Exchange in Critically Ill Children with Neuroimmunological Disorders: A Single-Center Cohort
Ebru G Sahin1, Kubra B Guvenc1, Gulcan A Yucel2
1Department of Pediatric Intensive Care, Sancaktepe Sehit Prof. Dr. Ilhan Varank Training and Research Hospital, University of Health Sciences, Istanbul 34785, Türkiye.
Insights
Therapeutic plasma exchange (TPE) shows promise for pediatric neuroimmunological disorders, with 63.6% of critically ill children achieving favorable outcomes. However, its use extends beyond current guidelines, necessitating further research for optimal application.
Area of Science:
- Pediatric Neurology
- Neuroimmunology
- Intensive Care Medicine
Background:
- Therapeutic plasma exchange (TPE) is increasingly utilized for pediatric neuroimmunological disorders.
- Evidence for TPE in rare neuroinflammatory and infection-triggered encephalopathy syndromes remains limited.
- This study examines TPE use in critically ill children within a tertiary pediatric intensive care unit.
Purpose of the Study:
- To describe clinical characteristics, treatment patterns, and outcomes of pediatric patients undergoing TPE for neuroimmunological disorders.
- To evaluate the effectiveness and safety of TPE in a diverse group of critically ill children.
- To identify gaps between clinical practice and evidence-based recommendations for TPE in pediatric neuroimmunology.
Main Methods:
- Retrospective observational study of pediatric patients (<18 years) receiving TPE for neuroimmunological disorders (Sept 2020 - Dec 2025).
- Data collected included demographics, disease spectrum, TPE indications, treatment details, neuroimaging, CSF analysis, and clinical outcomes.
- Neurological outcome assessed using Pediatric Cerebral Performance Category (PCPC) score at discharge.
Main Results:
- Twenty-two patients were included, with Guillain-Barré syndrome and autoimmune encephalitis being the most common diagnoses.
- The median interval to TPE initiation was 9 days, with a median of 7 TPE sessions.
- Favorable neurological outcomes were observed in 63.6% of patients; mortality was 13.6%, confined to severe encephalopathic syndromes. No major TPE complications were noted.
Conclusions:
- TPE is applied to a heterogeneous range of pediatric neuroimmunological disorders, including off-label indications.
- A significant proportion of cases fall outside current ASFA guidelines, indicating a gap in evidence.
- Multicenter studies are crucial to define optimal TPE use, timing, and patient selection in pediatric neuroimmunology.
Abstract:
Background: Therapeutic plasma exchange (TPE) is increasingly used in pediatric neuroimmunological disorders; however, evidence remains limited, particularly for rare neuroinflammatory and infection-triggered encephalopathy syndromes. We aimed to describe the clinical characteristics, treatment patterns, and outcomes of critically ill children who underwent TPE for neuroimmunological disorders in a tertiary pediatric intensive care unit. Methods: This retrospective observational study included consecutive patients younger than 18 years who received TPE for neuroimmunological disorders between September 2020 and December 2025. Demographic characteristics, disease spectrum, TPE indications, treatment details, neuroimaging findings, cerebrospinal fluid (CSF) characteristics, and clinical outcomes were reviewed. Neurological outcome at hospital discharge was assessed using the Pediatric Cerebral Performance Category (PCPC) score. Results: Twenty-two patients were included. The median age was 104 months (IQR, 62.3-151.8), and 50% were female. Guillain-Barré syndrome (GBS) was the most common diagnosis (n = 6), followed by autoimmune encephalitis (n = 6), infection-triggered encephalopathy syndrome (ITES)/acute necrotizing encephalopathy (ANE) spectrum disorders (n = 4), myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) (n = 3), acute disseminated encephalomyelitis (ADEM) (n = 2), and mild encephalitis/encephalopathy with a reversible splenial lesion (MERS) (n = 1). The median interval from symptom onset to TPE initiation was 9 days (IQR, 6-13), and patients underwent a median of 7 TPE sessions (IQR, 5-10.5). Nine patients (40.9%) required invasive mechanical ventilation and four (18.1%) required vasoactive support. Favorable neurological outcomes at hospital discharge were observed in 63.6% of patients, whereas 36.4% had unfavorable neurological outcomes. Mortality occurred in 13.6% of patients and was confined to severe encephalopathic syndromes. No major TPE-related complications were observed. Conclusions: TPE was used across a heterogeneous spectrum of pediatric neuroimmunological disorders, including both guideline-supported and non-standard indications. Approximately one-quarter of patients had conditions not specifically addressed in current ASFA recommendations, highlighting the gap between real-world clinical practice and the available evidence base. Multicenter studies are needed to better define the optimal role, timing, and patient selection criteria for TPE in pediatric neuroimmunology.