ASH 2021 guidelines on the management of von Willebrand disease

Recommendations include:

1. In patients with VWD with a history of severe and frequent bleeds, the guideline panel suggests using long-term prophylaxis rather than no prophylaxis.
2. In patients for whom desmopressin is a valid treatment option (primarily type 1 VWD) and who have a baseline VWF level of <0.30 IU/mL, the panel suggests performing a trial of desmopressin and treating based on the results over not performing a trial and treating with tranexamic acid or factor concentrate.
3. In these patients, the panel suggests against treating with desmopressin in the absence of desmopressin trial results.
4. In patients with VWD and cardiovascular disease who require treatment with antiplatelet agents or anticoagulant therapy, the panel suggests giving the necessary antiplatelet or anticoagulant therapy over no treatment.
5. The panel suggests targeting both FVIII and VWF activity levels of ≥0.50 IU/mL for at least 3 days after surgery.
6. The panel suggests against using only FVIII ≥0.50 IU/mL as a target level for at least 3 days after surgery.
7. In patients undergoing minor surgery or minor invasive procedures, the panel suggests increasing VWF activity levels to ≥0.50 IU/mL with desmopressin or factor concentrate with the addition of tranexamic acid over raising VWF levels to ≥0.50 IU/mL with desmopressin or factor concentrate alone .
8. The panel suggests giving tranexamic acid alone over increasing VWF activity levels to ≥0.50 IU/mL with any intervention in patients with type 1 VWD with baseline VWF activity levels of >0.30 IU/mL and a mild bleeding phenotype undergoing minor mucosal procedures.
9. The panel suggests using either hormonal therapy (combined hormonal contraception [CHC] or levonorgestrel-releasing intrauterine system) or tranexamic acid over desmopressin to treat women with VWD with heavy menstrual bleeding who do not wish to conceive.
10. The panel suggests using tranexamic acid over desmopressin to treat women with VWD and heavy menstrual bleeding who wish to conceive.
11. In women with VWD for whom neuraxial anesthesia during labor is deemed suitable, the panel suggests targeting a VWF activity level of 0.50 to 1.50 IU/mL over targeting an activity level of >1.50 IU/mL to allow neuraxial anesthesia.
12. The guideline panel suggests the use of tranexamic acid over not using it in women with type 1 VWD or low VWF levels (and this may also apply to types 2 and 3 VWD) during the postpartum period).