Hematology Advisor: What are other important recommendation for clinicians treating VWD patients?

Dr Lawrence: In regards to diagnostic evaluation for patients with VWD, judicious integration of both standardized bleeding assessment tools and repeat laboratory monitoring is recommended with careful consideration of the limitations of these tools and testing, depending on patient population-specific variables.

The decision to pursue genetic testing in patients with VWD at this point in time should be made carefully, with the understanding that causative mutations are less likely to be found in patients with mild type 1 VWD and low VWF.


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Hematology Advisor: What are remaining research needs in the area of VWD?

Dr Lawrence: The genetics leading to VWD and variation in VWF levels will likely be the next focus in the clinical research landscape. Our current treatment models do not generally rely on this testing for most patients with VWD, making it impractical to support widespread genetic testing in all patients. However, increasing knowledge in this area will make this an appealing means of predicting inheritance patterns and potentially improving our ability to predict bleeding risk in young patients who have not yet undergone major hemostatic challenges.

Acquired VWD [that is] secondary to other disease states — such as lymphoproliferative or myeloproliferative disorders and cardiovascular disease — presents a unique diagnostic and therapeutic challenge for hematology providers. These patients are often at increased risk for thrombosis in addition to the bleeding complications secondary to [acquired] VWD (aVWD), and thus therapeutic management often must account for this and can differ from traditional VWD treatment strategies. As [availability] and use of extracorporeal membrane oxygenation (ECMO) and left ventricular assist devices (LVAD) increases, this is a unique population for which aVWD diagnostic and therapeutic strategies should be explored.

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References

  1. Fogarty H, Doherty D, O’Donnell JS. New developments in von Willebrand disease [published online May 12, 2020]. Br J Haematol. doi: 10.1111/bjh.16681
  2. Centers for Disease Control and Prevention. Data and statistics on von Willebrand disease. https://www.cdc.gov/ncbddd/vwd/data.html. Updated on October 28, 2019. Accessed on June 10, 2020.
  3. Gill JC, Castaman G, Windyga J, et al. Hemostatic efficacy, safety, and pharmacokinetics of a recombinant von Willebrand factor in severe von Willebrand disease. Blood. 2015;126(17):2038-2046.
  4. Peyvandi F, Mamaev A, Wang JD, et al. Phase 3 study of recombinant von Willebrand factor in patients with severe von Willebrand disease who are undergoing elective surgery. J Thromb Haemost. 2019;17(1):52-62.
  5. American Society of Hematology. American Society of Hematology’s guidelines for venous thromboembolism and von Willebrand disease. https://www.ashclinicalnews.org/news/american-society-hematologys-guidelines-venous-thromboembolism-von-willebrand-disease. Published October 1, 2018. Accessed on June 10, 2020.