1. Therapeutic plasma exchange in children with acute autoimmune central nervous system disorders
Agnieszka Prytuła (1), Johan Vande Walle (1), Helene Verhelst (2),Stefaan Claus (3), Sunny Eloot (3), Annik De Jaeger (4), Joke Dehoorne (1), Ann Raes (1)
Department of (1) Paediatric Nephrology and Rheumatology (2) Paediatric Neurology and Metabolic Disease
(3) Haemodialysis Unit (4) Paediatric Intensive Care Unit at the Ghent University Hospital, Belgium
Background
Results
targeted therapy
plasma exchange
available
aspecific
can be commenced
without a delay
gender
diagnosis
age (y)
follow-up time and outcome F
neuromyelitis optica (anti-AQP4 neg.) 8
17 months; full recovery M
transverse myelitis 3
3 months; paraplegia
encephalitis with status epilepticus 2
9 months; moderate motor disability 12
died 11 days after start TPE
Bickerstaff’s brainstem encephalitis
12 months; full recovery
autoimmune paraneoplastic encephalitis (astrocytoma) 5
13 months; mild motor
and visual impairment
acute disseminated encephalomyelitis /transverse myelitis 6
10 months; full recovery
7 months; spastic paraplegia, neurogenic bladder
neuromyelitis optica
autoantibody-mediated encephalitis
acute disseminated encephalo-myelitis
paraneoplatic CNS involvement
removal of
auto-antibodies
and immuno-modulation
Pitfalls of plasmapheresis in small children:
IV access, haemodynamic instability, electrolyte disturbances, bleeding, catheter- related infections, allergic reaction
Objectives
to present a reproducible protocol for therapeutic plasma exchange (TPE) which can be used also in small children
to describe the efficacy and safety of an intensive TPE regimen in young children
Patients and Methods
Study design:
retrospective case series: 8 children included
diagnoses:
transverse myelitis (n= 3)
neuromyelitis optica (n= 1)
autoimmune paraneoplastic encephalitis (n= 1)
(autoimmune mediated) encephalitis (n= 2)
Bickerstaff’s brainstem encephalitis ( n= 1)
Indications for TPE
insufficient response to the standard or first-line therapies
and/ or
progressive weakness
(imminent) respiratory failure
104 plasma exchange sessions (6 to 27 per patient)
total treatment time: 6 to 72 days (median 14)
6 episodes of adverse events related to plasma exchange
- allergic reaction to fresh frozen plasma (n= 1)
- hypotension with SBP< 50 mmHg (n= 4)
- symptomatic hypocalcaemia with iCa 0.86 mmol/l (n= 1)
Clinical outcome
median length of PICU admission: 17 days (range: 5- 37)
one child died during PICU admission
marked clinical and/or radiological improvement after 5 plasma exchange sessions in all survivors
six children required revalidation
Spectra Optia® device, Terumo BCT
five sessions on consecutive days
clinical and/ or radiological assessment
weight < 20 kg and/or hematocrit < 30%
priming with packed red cells and 0.9% NaCl
anticoagulation: citrate dextrose solution (ACD-A)
replacement fluid:
2/3 4% albumin
1/3 fresh frozen plasma
Conclusions
Intensive plasma exchange is feasible even in small children with an acceptable rate of adverse events. We suggest that TPE should be applied without a delay in refractory demyelinating CNS disorders confirmed on the imaging.
In encephalitis with suspected autoimmune background TPE can be used as a diagnostic test in a situation where the results of antibody assays are not available.