Updated Recommendations for Treatment of von Willebrand Disease

1. For patients with vWD with a history of severe and frequent bleeding episodes, the expert panel suggests using long-term prophylaxis rather than no prophylaxis (conditional recommendation based on low certainty of evidence). It is noted that bleeding history and the need for prophylaxis should be regularly assessed for this purpose.
2. For patients for whom desmopressin administration is a suitable treatment modality (primarily type 1) and the baseline von Willebrand factor (vWF) level is < 0.30 IU/ml, the expert panel prefers conducting a trial treatment with desmopressin and subsequent treatment based on the results of this test over therapy with tranexamic acid or coagulation factor concentrates without performing this test (conditional recommendation based on very low certainty of evidence).
3. For these patients, the expert panel is also against the initiation of desmopressin therapy without a trial dose (conditional recommendation based on very low certainty of evidence).
4. For patients with cardiovascular diseases requiring antiplatelet or anticoagulant therapy, the expert panel recommends the administration of this therapy (conditional recommendation based on low certainty of evidence). During treatment, it is also important to regularly reassess the risk of bleeding.
5. For patients following major surgery, the expert panel recommends maintaining FVIII and vWF activity ≥ 0.50 IU/ml for at least 3 days post-operatively (conditional recommendation based on very low certainty of evidence).
6. The expert panel simultaneously does not recommend maintaining only FVIII activity ≥ 0.50 IU/ml (conditional recommendation based on very low certainty of evidence). The preferred option is therefore to maintain both FVIII and vWF activity above the specified level; specific target levels should be individualized based on the specific patient, type of surgery, and bleeding history.
7. For patients undergoing minor surgery or invasive procedures, the expert panel recommends increasing vWF activity to ≥ 0.50 IU/ml using desmopressin or coagulation factor concentrates in combination with tranexamic acid (conditional recommendation based on very low certainty of evidence).
8. Furthermore, for patients with vWD type 1 with a mild bleeding phenotype and baseline vWF activity ≥ 0.30 IU/ml undergoing minor mucosal procedures, it is recommended not to attempt increasing vWF activity to ≥ 0.50 IU/ml but rather to manage the patient only with tranexamic acid (conditional recommendation based on very low certainty of evidence).
9. For patients with severe menstrual bleeding who do not plan to conceive, hormonal therapy (hormonal contraception or intrauterine device) or tranexamic acid is recommended before administering desmopressin (conditional recommendation based on very low certainty of evidence).
10. For patients with severe menstrual bleeding who plan to conceive, administration of tranexamic acid is recommended over treatment with desmopressin (conditional recommendation based on very low certainty of evidence).
11. For patients where neuraxial anesthesia is suitable during childbirth, the expert panel recommends achieving vWF activity in the range of 0.50–1.50 IU/ml rather than aiming for an increase to ≥ 1.50 IU/ml (conditional recommendation based on very low certainty of evidence).
12. For women in the postpartum period, the use of tranexamic acid is recommended over non-use in cases of vWD type 1 or low vWF levels (also applicable for types 2 and 3; conditional recommendation based on low certainty of evidence).

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Source: Connell N. T., Flood V. H., Brignardello-Petersen R. et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv 2021; 5 (1): 301–325, doi: 10.1182/bloodadvances.2020003264.