2024/12/09 by W. BETBOUT, F. BEN LAKHAL, S. FEKIH SALEM +1
#Acquired von Willebrand syndrome #Diagnosis #Inhibitors #Von Willebrand factor
paper · doi:10.71776/pist-jims-189225
Acquired von Willebrand syndrome (AWS) is a rare bleeding disorder. Its prevalence remains unknown and probably underestimated because of the variability of clinical signs and the similarity of biological findings to constitutional von Willebrand disease (vWD). AWS usually occurs in adults without personal or family history of bleeding diathesis. Different mechanisms are involved in the pathophysiology of AWS which lead tocirculating von Willebrand factor (vWF) degradation or clearance. Clinically, the bleeding diathesis is variable and non-specific. Biological diagnosis is a challenge due to the heterogeneity of pathophysiological mechanisms, and the non-standardization of techniques used. Neutralizing antibodies against vWF are rarely detected in patients with AWS but remain essential.