Summary
Von Willebrand disease (VWD), the most common genetic bleeding disorder, is characterised
by a quantitative or qualitative defect of von Willebrand factor (VWF). Patients with
VWD suffer from mucocutaneous bleeding, of severity usually proportional to the degree
of VWF defect. In particular, gastrointestinal bleeding associated with angiodysplasia
is often a severe symptom of difficult management. This review focuses on the pathophysiology,
diagnosis and treatment of VWD-associated gastrointestinal angiodysplasia and related
bleeding.
Keywords
von Willebrand disease - bleeding - gastrointestinal angiodysplasia - therapy