Successful Desensitization of a Patient with Possible IgE-Mediated Anaphylactic Reaction to FVIII/VWF Concentrate.

Background: Type 3 von Willebrand disease (VWD) is a severe bleeding disorder with a prevalence of 1:1 million live births. There are several von Willebrand factor (VWF) replacement products used in the treatment of acute bleeding episodes or as prophylaxis. Patients who receive multiple transfusions have an increased risk of developing antibodies against these products. These antibodies can lead to life-threatening anaphylactic reactions. Previous studies have identified immune complex formation and complement activation as the trigger for anaphylaxis, rather than IgE. IgE-mediated anaphylaxis against VWF concentrates has not yet been published. Case: A 4-year-old female patient with type 3 VWD was referred to our hospital because of an anaphylactic reaction during FVIII/VWF concentrate (Immunate®) infusion. She had previously received FVIII/VWF concentrate (Haemate P®) infusions 8 times without any complications. She did not have antibodies against VWF and FVIII, and serum IgA level was normal. Since she needed factor replacement therapy as a result of a growing hematoma on her scalp, we performed skin prick and intradermal tests 2 days after the reaction. The prick test, with FVIII/VWF (Immunate), was negative, but the intradermal test was positive. We administered a 12-step desensitization protocol with FVIII/VWF concentrate (Immunate) successfully without any reactions.
Conclusion: Anaphylactic reaction to factor replacement products is a major problem for patients with VWD, especially type 3 VWD requiring multiple factor infusions. We achieved a successful desensitization with FVIII/VWF concentrate in a patient who had an anaphylactic reaction during the infusion of this product. Our patient is important since she represents the first case of IgE-mediated anaphylaxis against VWF concentrate reported in the literature.