Prophylactic recombinant factor VIIa administration to an infant with congenital systemic juvenile xanthogranuloma

We report the case of an infant affected with congenital systemic juvenile xanthogranuloma scheduled for central venous access system implantation (Port-a-Cath(TM)) and a liver and bone marrow biopsy. The patient had impaired liver function, thrombocytopenia, and coagulopathy which was refractory to daily fresh-frozen plasma and platelet infusions: 80 mu g.kg(-1) dose(-1) of recombinant factor VIIa (rFVIIa) was administered i.v. every 2 h starting 30 min before the procedure and ending 6 h afterwards. Very minor bleeding was observed during the procedure. In conclusion, rFVIIa therapy was effective as prophylaxis for both invasive procedures in this patient with a coagulopathy which was refractory to other different therapies.