von Willebrand Disease: Management

von Willebrand Disease: overview

This algorithm summarizes management of von Willebrand disease, with disease type as the main branch point.

For mild type 1 disease, desmopressin (DDAVP) is first-line in patients who are DDAVP-responsive. For type 2 or type 3 disease, or for patients who do not respond to desmopressin, replacement with von Willebrand factor (VWF) concentrate is used, with or without factor VIII. Tranexamic acid, an antifibrinolytic, serves as an adjunct, particularly for mucosal bleeding. The decision therefore rests on type and documented desmopressin responsiveness: patients who can release enough endogenous VWF are managed with desmopressin, while those with qualitative defects, severe deficiency or an inadequate response require factor replacement, and antifibrinolytic therapy can be layered onto either approach.

Background reading: Von Willebrand Disease (vWD) board review notes.