Area of Science:

• Hematology
• Internal Medicine
• Pathology

Background:

• Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder.
• It presents similarly to inherited von Willebrand disease but without personal or family history of bleeding.
• AVWS predominantly affects elderly individuals, with mucocutaneous bleeding being the most common manifestation.

Purpose of the Study:

• To review the clinical presentation, underlying causes, pathophysiology, diagnostic challenges, and management of acquired von Willebrand syndrome.
• To highlight the importance of recognizing AVWS in the differential diagnosis of bleeding disorders, particularly in the elderly population.

Main Methods:

• Literature review of acquired von Willebrand syndrome.
• Analysis of clinical features, laboratory findings, and underlying conditions associated with AVWS.
• Discussion of diagnostic approaches and therapeutic strategies.

Main Results:

• Commonly associated conditions include monoclonal gammopathies, lymphoproliferative disorders, autoimmune diseases, and cardiovascular pathologies.
• Pathophysiology involves autoantibodies against von Willebrand factor (vWF), increased proteolysis, mechanical destruction, or adsorption onto tumor cells/activated platelets.
• Diagnosis is often challenging due to the need for multiple laboratory tests.

Conclusions:

• Acquired von Willebrand syndrome requires a thorough investigation to identify underlying pathologies.
• Management focuses on treating the primary condition and preventing bleeding events.
• Early recognition and appropriate diagnostic workup are crucial for effective patient care.