In patients with moderate or mild haemophilia A, administration of desmopressin (Minirin®) may suffice to ensure adequate levels of factor VIII (i.e. 2 to 8 times base level)).
Prophylaxis and treatment involve the administration of plasma-derived or recombinant factor VIII concentrates.
In the presence of anti-factor VIII antibodies, recombinant factor VIIa may be used as well as activated prothrombin complex concentrates such as Feiba® or Autoplex®.
Other therapeutic measures may be adopted in certain specific situations. The activity level for factor VIII needed to ensure clotting is around 30%. (1) Factor Eight Inhibitor Bypassing Activity
Monitoring involves measurement of factor VIII activity levels, as well as screening for, and assay of, specific inhibitors.
In patients treated with recombinant activated factor VII, the activity of this substance should be measured.
Recent studies have shown that global coagulation tests such as the thrombin generation test may constitute useful and effective tools for the adaptation of replacement therapy in patients presenting an inhibitor.
Other studies have demonstrated the value of thrombin generation testing in the preoperative workup for haemophilia A patients.