Acquired von Willebrand disease

Area of Science:

• Hematology
• Internal Medicine
• Pathology

Background:

• Acquired von Willebrand disease (AvWD) is a rare bleeding disorder.
• It often occurs without a prior history of bleeding issues.
• AvWD is frequently associated with monoclonal gammopathies, lymphoproliferative, myeloproliferative, and autoimmune disorders, and less commonly with drugs or solid tumors.

Purpose of the Study:

• To review the pathogenesis and treatment of acquired von Willebrand disease.
• To highlight the diverse mechanisms leading to von Willebrand factor (vWF) deficiency or dysfunction.
• To outline current therapeutic strategies for managing AvWD.

Main Methods:

• Literature review of acquired von Willebrand disease.
• Analysis of pathogenetic mechanisms including autoantibodies, vWF clearance, adsorption, proteolysis, decreased synthesis, and precipitation.
• Summary of treatment options such as addressing underlying disorders, vWF concentrate infusion, desmopressin (DDAVP), intravenous gammaglobulin, plasma exchange, and immunoadsorption.

Main Results:

• Multiple pathogenetic mechanisms contribute to AvWD.
• Autoantibodies against von Willebrand factor (vWF) are a key factor, leading to rapid vWF clearance or inactivation.
• Other mechanisms include vWF adsorption to malignant cells, drug-induced proteolysis, decreased synthesis, and precipitation.

Conclusions:

• Effective management of AvWD requires addressing the underlying disorder when possible.
• Symptomatic treatment involves replacing vWF via concentrates or DDAVP.
• Immunomodulatory therapies like IVIg, plasma exchange, or immunoadsorption can be beneficial in cases with anti-vWF antibodies.