Successful desensitization with FVIII/Von Willebrand Factor concentrate in Type III Von Willebrand Disease

Von Willebrand Disease (VWD) Type 3 is a rare and severe bleeding disorder characterized by an almost complete deficiency of Von Willebrand Factor (VWF). Plasma-derived Factor VIII (FVIII)/VWF concentrates are used both on demand and for prophylactic treatment. However, allergic reactions to these products pose significant challenges in clinical management. A 40-year-old female patient with VWD Type 3 presented to our clinic with symptoms including jaw numbness and tightness, chills, fatigue, nausea, and dyspnea following administration of a FVIII/VWF concentrate (Haemate^® P). Skin prick tests and intradermal tests were performed for diagnostic evaluation and were found to be negative. The hypersensitivity reaction was assessed as an immediate type and non-IgE-mediated reaction. As there were no alternative treatment options, we decided to perform desensitization. A 14-step desensitization protocol was successfully administered. The patient is now able to self-administer Haemate^® P at home three times a week. This case highlights the importance of desensitization and multidisciplinary approach in the case of drug hypersensitivity in patients with VWD Type 3. Our desensitization protocol with FVIII/VWF concentrate is highly effective and safe.