Objective The aim of our study was to determine the outcome of children with severe life-threatening symptoms treated with plasmapheresis alone or with plasmapheresis and immunosuppression. The goal of plasmapheresis is either removing antibodies or immune complexes or substitution of a missing protein.
Methods 12-bed interdisciplinary paediatric intensive care unit.
Patients 16; 6 male, 10 female; mean age 12.1 years, treatment with plasmapheresis: 1–18 mean 5.5. Indications for plasmapheresis by diagnoses with severe organ failure: nephrological patients, 8; neurological patients, 6; other, 2. Additional immunosuppression consisted of steroids, mycophenolate mofetil, cyclophosphamide and rituximab.
Results The neurological group consisted of three patients with severe myasthenia gravis, one with chorea minor, and two with chronic inflammatory demyelinating neuropathy. The nephrological group consisted of three patients with atypical haemolytic uraemic syndrome (HUS) and five patients with lupus nephritis with vascular lesions; one patient had hyperproteinemia with renal failure, one had autoantibodies against thrombocytes after bone marrow transplantation. All neurological patients improved and stayed in remission with immunosuppression for more than 3 years. Five of the nephrological patients (3 lupus erythematosus (LE), 2 HUS) improved and stayed in remission for 3 years. One patient with LE was transplanted successfully, one patient with LE with non-compliance is on haemodialysis, and one patient with HUS improved. The patient with hyperproteinemia recovered completely, the patient with autoantibodies against thrombocytes died.
Conclusion In paediatric patients with severe organ failure resulting from a diagnosis indicating plasmapheresis, combined treatment with plasmaphereses and immunosuppresion led to persistent remission of organ failure.