Major surgery
This section includes a recommendation for targeting both factor VIII and VWF activity levels to a minimum of 50 IU/mL for at least 3 days after surgery, and a suggestion against using factor VIII target levels alone.
Minor surgery/invasive procedures
The panelists suggest increasing VWF activity levels to a minimum of 0.50 IU/mL with desmopressin or factor concentrate with the addition of tranexamic acid over raising VWF levels to at least 0.50 IU/mL with desmopressin or factor concentrate alone.
In addition, the panelists suggest “giving tranexamic acid alone over increasing VWF activity levels to a minimum threshold of 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.”
Heavy menstrual bleeding
In women with heavy menstrual bleeding who do not plan to conceive, the panel suggests either combined hormonal therapy or levonorgestrel-releasing intrauterine system, or tranexamic acid over desmopressin.
In women who wish to conceive, the panel suggests using tranexamic acid over desmopressin.
Neuraxial anesthesia during labor
For women in labor for whom neuraxial anesthesia is considered, the guidelines suggest targeting a VWF activity level from 0.50 to 1.50 IU/mL over targeting a level above 1.50 IU/mL.
Postpartum management
“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,” the guidelines say.
An accompanying good practice statement says that tranexamic acid can be provided orally or intravenously. The oral dose is 25 mg/kg three times daily for 10-14 days, or longer if blood loss remains heavy.
Dr. Flood said that the guidelines were developed under the assumption that they would apply to care of patients in regions with a high or moderately high degree of clinical resources.
“We recognize that this eliminates a great deal of the globe, and our hope is that ASH and the other sponsoring organizations are going to let us revise this and do a version for lower-resourced settings,” she said.