The treatment of bleeding in acquired haemophilia with recombinant factor VIIa: a multicentre study.

摘要: Recombinant factor VIIa was used to treat 38 patients with acquired haemophilia participating in the Novoseven compassionate-use program. 19 were male, median age 59, range 2-89 years. The pre-treatment anti-human (H) and anti-porcine (P) inhibitor titre H 43 BU/ml (range 1-4500) P 4.5 0-1600). VIIIa as first-line therapy for 14 bleeding episodes salvage-therapy 60 which failed respond blood-product given a of four days 1-21 days) prior treatment rVIIa. Pre-rVIIa not reported episodes. indications 7 haemarthroses, 40 muscle haematomas, 20 urinary or GI haemorrhages 3 surgical interventions. starting dose rVIIa 90.4 microg/kg 45-181). A 28 doses 1-541) per episode, over 3.9 0-43). Efficacy assessed clinically 8 24 h after start at end treatment. good response obtained all bleeds rVIIIa therapy. 75%, partial 17% poor 8%. unreported 4 cases. prothrombin time (PTT) shortened from 12 s 9.3-20) 8.8 6-14) during clinical did correlate used, type bleed degree shortening PTT following infusion. Three died haemorrhagic complications haemophilia. This mortality 7.9% is lower than previously this condition. Although one patient developed DIC rVIIa, probably attributable hypovolaemic shock, massive transfusion use PCCs. study demonstrates that safe, useful effective