Management of patients with alloantibodies to VWF

Although very seldom, some type 3 VWD patients develop anti-VWF alloantibodies after multiple transfusions. Exposure to VWF containing products may cause life-threatening post-infusion anaphylaxis besides being ineffective. 

• Recombinant FVIII can be given at large doses during surgery. Continuous infusion is mandatory due to the very short half -life of FVIII.

• By-passing therapy with activated prothrombin complex concentrates aPCC (FEIBA) and rFVII (NovoSeven) may also be considered. 

• IV immunoglobulins prior to administering VWF may help to achieve critical levels of VWF and FVIII.