Unresponsiveness to factor VIII inhibitor bypassing agents during haemostatic treatment for life-threatening massive bleeding in a patient with haemophilia A and a high responding inhibitor.

摘要: Summary.   We report a case of haemophilia A with high responding inhibitor factor VIII (FVIII) who had serious retroperitoneal haematoma caused by penetration duodenal ulcer. Inhibitor-bypassing therapy was commenced immediately on admission. On the 17th day treatment activated prothrombin complex concentrate (APCC; FEIBA®), re-bleeding occurred and thrombelastography (TEG) demonstrated resistance to therapy. Treatment changed recombinant VII (rFVIIa; NovoSeven®) resulted in clinical improvement together an TEG parameters. 10th continuous infusion NovoSeven®, however, again showed FEIBA® infusions were re-introduced results remained satisfactory for 7 days. 34, further bleeding evident decline haemostatic efficiency recorded TEG. NovoSeven® successfully administered There no laboratory findings indicate disseminated intravascular coagulation (DIC), hypercoagulability or abnormal fibrinolysis. The plasma-based clotting tests did not show any additional prolongation occasions when unresponsiveness NovoSeven®. These suggested that some component whole blood, other than plasma might have governed data. long-term use APCC such as rFVIIa, requires careful monitoring terms FVIII bypassing activity well tendency DIC.