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Treatment of intracranial and extracranial haemorrhages in a neonate with severe haemophilia B with recombinant factor IX infusion.

Haemophilia. 2005 Jul;11(4):411-4.

• Guilcher GM, Scully MF, Harvey M, Hand JP.

Department of Paediatrics, Janeway Children's Health and Rehabilitation Centre, Memorial University of Newfoundland, St John's, NL, Canada. gguilcher@cw.bc.ca

Intracranial (ICH) and extracranial (ECH) haemorrhages are potentially life-threatening events that may occur comorbidly in neonates with haemophilia. There is little data on the use of recombinant factor IX (rFIX; BeneFIX in the neonate. Children <15 years of age are known to require higher doses of recombinant Factor IX (FIX) than older persons, which raises specific concerns in the neonate due to the increased risk of thrombosis in this age group (Thromb Haemost 2002; 87: 431). This report describes a case in which a high rate of continuous infusion of recombinant FIX was used to treat a newborn with significant intracranial and subgaleal haemorrhages. A high rate of infusion maintained at 30-35 U kg(-1) h(-1) was necessary to maintain adequate FIX levels. Despite the high rate of continuous infusion, no adverse events were noted. Our patient had a rare genetic mutation causing severe haemophilia B. A neonate with severe haemophilia B was treated successfully with recombinant FIX through continuous infusion. A high rate of infusion was required and no complications were noted.

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