Last update:
29-Sep-2020

Acquired von Willebrand syndrome (AVWS) is a hemorrhagic manifestation of a heterogeneous group of diseases in patients with a negative personal and family history of bleeding diathesis. Cardiovascular diseases, lymphoproliferative and myeloproliferative syndromes, autoimmune disorders, solid tumors, and various pharmaceuticals are the most commonly associated inductive factors. Its frequency is often underestimated, because of the mildness of the hemorrhagic events, and its diagnostic difficulty, requiring specialized laboratory tests. The pathophysiology involves a variety of biological mechanisms, even in the setting of the same pathological entity. The basic pathogenetic pathways include the inactivation of von Willebrand factor (VWF) by autoantibodies, increased clearance of VWF due to the development of immune complexes and high shear stress, and reduced VWF synthesis and release. AVWS usually combines laboratory and clinical findings similar to those in the type 2 form of the hereditary disease. Diagnosis requires a series of tests to assess the antigenic and functional VWF levels, the coagulation factor VIII, and the VWF high molecular weight polymers. Treatment of the underlying disorder leads to the reverse of the VWF alterations and regression of symptoms. Desmopressin and VWF concentrates are used, among other drugs, to control or prevent active hemorrhagic events in high-risk patients. This review summarizes the currently available data regarding the pathophysiology, laboratory investigation, and management of this hemostatic disorder.

Key words: Αcquired von Willebrand syndrome, Diagnosis, Pathophysiology, Therapy.