Managing acute bleeds in the patient with haemophilia and inhibitors: options, efficacy and safety.
The options available for treating the patient with haemophilia and inhibitors undergoing surgery or with other acute bleeds include high-dose factor VIII (FVIII) (human or porcine), prothrombin complex concentrates (PCCs), activated PCCs (aPCCs), recombinant activated factor VII (rFVIIa), and factor replacement combined with immunoadsorption or immunosuppression. Human FVIII is effective in patients with low-titre inhibitors. Porcine FVIII is currently not available, and PCCs and aPCCs, although effective, have been associated with a high incidence of adverse events. Immunoadsorption and immunosuppression offer excellent long-term solutions, but the duration of these techniques makes them less attractive for use in acute settings. Recombinant FVIIa has demonstrated excellent efficacy and safety, even in patients refractory to other therapies.
von Depka
M
M
Werlhof-Institute for Haemostasis and Thrombosis, Karl-Wiechert-Allee, Hannover, Germany. mvd@werlhof-institut.de
D000208:Acute Disease; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood*; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000627:therapeutic use; D015942:Factor VIIa; D006467:Hemophilia A / Q000150:complications / Q000628:therapy*; D006470:Hemorrhage / Q000209:etiology / Q000628:therapy*; D006801:Humans; D007163:Immunosorbent Techniques; D007165:Immunosuppression Therapy / Q000379:methods; D008297:Male; D011994:Recombinant Proteins / Q000627:therapeutic use
D019774:Blood Coagulation Factor Inhibitors; D011994:Recombinant Proteins; D005167:Factor VII; D005169:Factor VIII; C103587:recombinant FVIIa; D015942:Factor VIIa