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 language | English |
|---|---|
| Pages (from-to) | 518-521 |
| Number of pages | 4 |
| Journal | Blood Coagulation and Fibrinolysis |
| Volume | 25 |
| Issue number | 5 |
| DOIs | |
| Publication status | Published - 2014 |
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Keywords
- Cyclophosphamide
- Haemophilia
- Immunotolerance induction therapy
- Inhibitors
- Intracranial haemorrhage
- Neurosurgery
- Recombinant-activated FVII
- Retuximab
ASJC Scopus subject areas
- Hematology
- Medicine(all)
Cite this
Successful urgent neurosugery management with rFVIIa mega doses in a child with haemophilia A and high titre inhibitor. / Banov, Laura; Pavanello, Marco; Piattelli, Gianluca; Disma, Nicola; Severino, Mariasavina; Dufour, Carlo; Molinari, Angelo Claudio.
In: Blood Coagulation and Fibrinolysis, Vol. 25, No. 5, 2014, p. 518-521.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Successful urgent neurosugery management with rFVIIa mega doses in a child with haemophilia A and high titre inhibitor
AU - Banov, Laura
AU - Pavanello, Marco
AU - Piattelli, Gianluca
AU - Disma, Nicola
AU - Severino, Mariasavina
AU - Dufour, Carlo
AU - Molinari, Angelo Claudio
PY - 2014
Y1 - 2014
N2 - 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.
AB - 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.
KW - Cyclophosphamide
KW - Haemophilia
KW - Immunotolerance induction therapy
KW - Inhibitors
KW - Intracranial haemorrhage
KW - Neurosurgery
KW - Recombinant-activated FVII
KW - Retuximab
UR - http://www.scopus.com/inward/record.url?scp=84905007522&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84905007522&partnerID=8YFLogxK
U2 - 10.1097/MBC.0000000000000074
DO - 10.1097/MBC.0000000000000074
M3 - Article
VL - 25
SP - 518
EP - 521
JO - Blood Coagulation and Fibrinolysis
JF - Blood Coagulation and Fibrinolysis
SN - 0957-5235
IS - 5
ER -