Successful urgent neurosugery management with rFVIIa mega doses in a child with haemophilia A and high titre inhibitor

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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.

Original languageEnglish
Pages (from-to)518-521
Number of pages4
JournalBlood Coagulation and Fibrinolysis
Volume25
Issue number5
DOIs
Publication statusPublished - 2014

Fingerprint

Hemophilia A
Neurosurgery
Hemorrhage
Costs and Cost Analysis
Appointments and Schedules
recombinant FVIIa
Therapeutics

Keywords

  • Cyclophosphamide
  • Haemophilia
  • Immunotolerance induction therapy
  • Inhibitors
  • Intracranial haemorrhage
  • Neurosurgery
  • Recombinant-activated FVII
  • Retuximab

ASJC Scopus subject areas

  • Hematology
  • Medicine(all)

Cite this

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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.",
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AU - Disma, Nicola

AU - Severino, Mariasavina

AU - Dufour, Carlo

AU - Molinari, Angelo Claudio

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