rVIIa therapy to secure haemostasis during central line insertion in children with high-responding FVIII inhibitors.
The provision of adequate haemostasis in children with haemophilia A (HA) who have developed high-responding inhibitors continues to be a great challenge to haematologists and institutional resources. The most exciting development in the management of acute bleeding in these patients, irrespective of inhibitor titre, has been the use of recombinant activated factor VII (rVIIa). Three severe HA children with high-responding inhibitors underwent four central line insertions (two Hickman catheters and two port-a-caths, one replaced because of infection) with rFVIIa cover to enable continuous FVIII infusions. No adverse haemorrhagic events occurred and immune tolerance therapy (ITT) using high-dose FVIII therapy was initiated and inhibitor eradication was achieved in al three patients at 8, 10 and 7 months. A further patient who had a central line in situ prior to the development of a high-responding inhibitor was also successfully 'tolerized' at 6 months using the same protocol. Interestingly, all four patients had the IVS 22 mutation.
Smith
O P
OP
Institute of Child Health, London, England.
Hann
I M
IM
D017668:Age of Onset; D002405:Catheterization, Central Venous; D002408:Catheters, Indwelling; D005169:Factor VIII / Q000037:antagonists & inhibitors*; D015942:Factor VIIa / Q000008:administration & dosage / Q000627:therapeutic use*; D006467:Hemophilia A / Q000628:therapy*; D006487:Hemostasis; D006801:Humans; D007223:Infant; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use
D011994:Recombinant Proteins; D005169:Factor VIII; D015942:Factor VIIa