Therapeutic plasma exchange in paediatric neurology: indications, side effects and outcomes from four UK tertiary centres

INTRODUCTION: Therapeutic plasma exchange (TPE) is increasingly used in a range of neurological illnesses in children characterised by the presence of autoantibodies or suspected immune dysregulation.  Our aim was to ascertain the indications, side effects and outcomes for children receiving TPE.  METHODS: Multicentre retrospective case review of 58 paediatric neurology patients undergoing TPE from 2005-2013.  Outcome was assessed using modified Rankin Scale (mRS) for children (http://www.neuropaediatrie.ch/snpsr/Modified%20Rankin%20Scale.pdf).  RESULTS: Median age at treatment was 9.2 years (range 1.3-16.0) with 23 days elapsed since onset of neurological symptoms (range 1 day-12.5 years).  Indications included transverse myelitis (n=16), ADEM (n=9), myasthenia gravis (n=8), NMDAR encephalitis (n=7), Guillain-Barre syndrome (n=5), CIDP (n=4), VGKC encephalitis (n=3) and FIRES (n=2).  A median 2 treatments were tried before TPE (range 0-4) including steroids (88%) and IVIG (70%).  Courses comprised a median 6 exchanges (range 2-179) given over 8 days (range 3-466).  36% of courses were initiated in PICU.  Reported complications included hypocalcaemia (n=13), suspected line infection (n=8), anaemia (n=6), coagulopathy (n=5) and hypotension (n=5).  73% of patients were severely disabled (mRS=5) at treatment initiation.  There was a statistically significant improvement in mRS score immediately following treatment, with improvement in 21% of patients and no change in 79% (Wilcoxon signed-rank test, p<0.001).  Treating clinician’s impression of effect was positive in 65%.  Median mRS at follow-up was 3 (moderate disability); 9% were severely disabled (n=4) or dead (n=1).  DISCUSSION: This study provides safety and efficacy information on TPE for clinicians and families and provides a basis for future prospective studies.