title: Successful urgent neurosugery management with rFVIIa mega doses in a child with haemophilia A and high titre inhibitor.
abstract: We report an urgent aggressive neurosurgery procedure for a large life-threatening intracranial bleed in a 3-year-old boy with severe haemophilia A and high titre inhibitor, managed with mega doses of recombinant-activated FVII (rFVIIa). We infused preoperatively bolus of 350 μg/kg, repeated every 2 h for 4 days. There were no bleeding complications during surgery. Afterward, rFVIIa was gradually tapered acting alternatively on dose and timing, until the ongoing schedule of 214 μg/kg every 12 h.To our knowledge this is the first report of aggressive neurosurgery in a boy with high titre inhibitor, successfully managed with high doses rFVIIa.The close and prompt collaboration between haematologist, neurosurgeon, and anaesthesiologist was successful in managing the critical haemorrhage without major sequelae and eradicating the inhibitor, at a cost of about 1.500.000 Euros. There is an urgent need for availability of standardized global assay to monitor the rFVIIa treatment, which could contribute to constrain these prohibitive costs.
authors: aThrombosis and Haemostasis Unit, Department of Translational Research and Laboratory Medicine bNeurosurgery, Surgery Department cNeuroradiology Unit dIntensive Care Unit, Department of Emergency and Intensive care eClinical and Experimental Haematology Unit, Department of Haematology, Oncology and Stem Cell Transplantation, Giannina Gaslini Children's Hospital, Genova, Italy.
authors: Laura
authors: L
authors: Banov
authors: Marco
authors: M
authors: Pavanello
authors: Gianluca
authors: G
authors: Piattelli
authors: Nicola
authors: N
authors: Disma
authors: Mariasavina
authors: M
authors: Severino
authors: Carlo
authors: C
authors: Dufour
authors: Angelo Claudio
authors: AC
authors: Molinari
mesh_terms: D002675:Child, Preschool; D019468:Disease Management; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy* / Q000601:surgery*; D006801:Humans; D007518:Isoantibodies; D008297:Male; D009493:Neurosurgery / Q000379:methods*; D011994:Recombinant Proteins / Q000008:administration & dosage / Q000627:therapeutic use
chemical_list: D007518:Isoantibodies; D011994:Recombinant Proteins; C103587:recombinant FVIIa; D015942:Factor VIIa