Ezio Zanon

Objectives The present review aims to summarize the state‐of‐the‐art von Willebrand disease (VWD) treatment focusing on specific clinical settings (obstetrics, surgery, long‐term prophylaxis and comorbidities) as well as on the use of a Von Willebrand factor (VWF) concentrate with low FVIII content. Methods Literature research and case reports. Results and Conclusions Considering that patients affected by VWD have an intact ability to synthesize FVIII, in order to avoid excessive levels of FVIII, a highly purified plasma VWF concentrate with low FVIII content could be particularly useful in those patients and clinical circumstances at high thrombotic risk as well as for long‐term prophylaxis. When deciding the optimal therapeutic strategy, physicians should take into account both the patient's history and the differences among available concentrates according to the clinical situations requiring treatment.