Therapeutic target
Hemostasis or hemorrhage prophylaxis.
Therapy recommendations
- See specifics for the following agents depending on the type of Willebrand-Jürgens syndrome.
- Caution. Acetylsalicylic acid (ASA) is contraindicated in patients with Willebrand-Jürgens syndrome.
| Type | Active ingredients | Special features |
| 1 + 2 | Desmopressin | For light bleeding |
| 3 | Desmopressin | Continuous infusion during surgery (OP) |
| Factor VIII/von Willebrand factor concentrate | In cases of severe bleeding and for prophylaxis before surgery | |
| Tranexamic acid | Additionally in case of mucosal bleeding,- surgery. |
Further notes
- Under certain circumstances, prophylactic substitution therapy is indicated in von Willebrand-Jürgens syndrome type 3 (factor VIII/vWF concentrate 20 IU/kg bw 2x/week).
