ABSTRACT 604

Abstract

Background and aims: Plasmapheresis (PF) is an extracorporeal blood purification technique designed for the removal of large molecular weight substances. The basic premise of the treatment is that the removal of these substances will allow for the reversal of the pathologic processes related to their presence. PF has seen an increase in usage in critically ill patients with immune dysregulation. Aims: To review the indications, number of procedures, morbidity and clinical evolution of PF in critically ill children with autoimmune diseases. Methods: Prospective study in a 8 bed PICU. A discontinuous flow centrifugation system was used (Haemonetics). Diagnosis, demographic, ICU days, outcome and complications of the technique were determined. OFI score (max score 6) was measured daily. Values are expressed in median (range). Results: 32 PF procedures on 6 patients with age 92m (48 -168), weight 34 kg(16–70), OFI score of 5(3–6) and Ferritin levels of 16803 μg/dL (1700–39000) were reviewed. The diagnosis were macrophage activation syndrome 2, hemophagocytic lymphohistiocytosis 1, anti NMDAR encephalitis 1, TPO encephalitis 1 and acute disseminated encephalomyelitis 1. All received previously metilprednisolone and immunoglobulin, 2 cyclosporine and 1 rituximab. The procedure was performed 3 days (0–5) after PICU admission. The serum ferritin and OFI score decreased after 2 PF sessions.The most frequent complication, was hypotension requiring fluid bolus in 15 procedures .5 patients survived, after a mean of 17 days (1–52) in the PICU. Conclusions: PF should be considered as rescue treatment in pediatric patients with life threatening autoimmune diseases refractory to conventional therapies.