Current Recommendations of the Expert Panel on the Management of von Willebrand Disease
Von Willebrand disease (vWD) is a common congenital bleeding disorder. The care of patients with vWD involves significant variability due to bleeding manifestations and treatment options. The new recommendations from 2021 regarding the care of people with vWD are evidence-based and supported by the American Society of Hematology (ASH), the International Society on Thrombosis and Haemostasis (ISTH), the National Hemophilia Foundation USA (NHF), and the World Federation of Hemophilia (WFH). We bring you a concise summary of the key points.
The expert panel agreed on several clinical practice recommendations that it considers important for discussion. They also indicate directions and possibilities for future research.
- For patients with vWD and a history of frequent or severe bleeding, long-term prophylaxis is recommended rather than no prophylaxis. It is also important to regularly reassess bleeding manifestations.
- For patients with vWD for whom desmopressin (DDAVP) administration is feasible, a DDAVP challenge test is recommended (with subsequent management guided by the test result) rather than not performing the test. This is particularly relevant for patients with type 1 vWD and von Willebrand factor (vWF) levels < 0.30 IU/ml. Simultaneously, the panel recommends not treating with desmopressin if the test has not been performed beforehand. This recommendation does not apply to patients for whom DDAVP is not a practical treatment option (especially those with type 3 vWD and contraindications in type 2B). Many patients with type 2 vWD do not respond to DDAVP, but testing is possible. For individuals with type 1 vWD and vWF levels > 0.30 IU/ml, a response to DDAVP is expected, although testing is still recommended.
- For patients with vWD and cardiovascular disease requiring antiplatelet or anticoagulant therapy, administering such treatment rather than withholding it is recommended. However, it is important to carefully assess the bleeding risk during such therapy.
- For major surgeries, maintaining FVIII and vWF activity levels above 0.50 IU/ml for at least the first 3 postoperative days is recommended. Therefore, the panel does not recommend solely monitoring FVIII levels maintained above 0.50 IU/kg. Specific safe target levels may be individualized based on the type of procedure, patient history, and testing capabilities of vWF and FVIII levels.
- For patients with vWF undergoing minor invasive or surgical procedures, increasing vWF activity above 0.50 IU/ml using desmopressin or vWF concentrate along with tranexamic acid administration is recommended rather than withholding it. For patients at higher risk of thrombotic complications, avoiding prolonged combinations of elevated vWF and FVIII levels (> 1.50 IU/ml) with extended tranexamic acid administration is advisable.
- For women with significant menstrual bleeding, either hormonal therapy (combined oral or intrauterine releasing levonorgestrel) or tranexamic acid is recommended. In this indication, tranexamic acid administration is preferred over desmopressin. Wherever possible, forming a multidisciplinary team, including a gynecologist and hematologist, to find the optimal care pathway for these patients is recommended.
- For women with vWD requiring neuraxial anesthesia during childbirth, targeting vWF levels between 0.50 and 1.50 IU/ml for safe procedure performance rather than achieving levels > 1.50 IU/ml is recommended.
- For women with type 1 vWD or low vWF levels in the postpartum period, oral tranexamic acid administration at a dose of 25 mg/kg 3 times daily or intravenously is recommended. The duration of administration is 10−14 days or longer if blood loss remains significant during the puerperium.
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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 Jan 12; 5 (1): 280−300, doi: 10.1182/bloodadvances.2020003265.
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