How I treat type 2B von Willebrand disease

Rebecca Kruse-Jarres and Jill M. Johnsen


Type 2B von Willebrand disease (VWD) is an inherited bleeding disorder caused by changes in von Willebrand factor (VWF) that enhance binding of VWF to GPIb on platelets. Although this disorder is seemingly well defined due to this single molecular defect, in reality type 2B VWD is a clinically heterogeneous disorder that can be difficult to identify and manage. Diagnostic criteria include a history of mucocutaneous bleeding, laboratory studies showing enhanced VWF binding of platelets and/or a 2B VWD genetic variant, and a family history consistent with autosomal dominant inheritance. Thrombocytopenia, while not always present, is common and can be exacerbated by physiologic stressors such as pregnancy. The mainstay of therapy for type 2B VWD is VWF replacement therapy. Adjunct therapies useful in other types of VWD, such as antifibrinolytics, are also used in type 2B VWD. Desmopressin (DDAVP) is controversial due to exacerbation of thrombocytopenia, but is in practice sometimes used for minor bleeding. Herein we review the available evidence and describe three clinical cases to illustrate the intricacies of diagnosing type 2B VWD, to describe the response to DDAVP and to review the complexities and management during pregnancy.

  • Submitted June 26, 2017.
  • Accepted January 4, 2018.