Acquired von Willebrand syndrome

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  • 後天性フォンヴィレブランド症候群
  • コウテンセイ フォンヴィレブランド ショウコウグン

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Abstract

<p>Von Willebrand disease (VWD) is among the most common inherited bleeding disorders. Interestingly, acquired von Willebrand syndrome (AVWS) is diagnosed much less frequently, but can be identified in association with a substantial number of medical conditions and diseases, including lymphoproliferative (48%), cardiovascular (21%), myeloproliferative (15%), other neoplastic (5%), and autoimmune disorders (2%). Most recently, AVWS has been diagnosed in patients with aortic valve stenosis (AS, 79%) and continuous-flow left ventricular assist devices (LVAD, up to 100%).</p><p>1) Immune mechanisms mediated reduction of VWF activity</p><p>Autoantibodies to VWF have been identified in association with monoclonal gammopathies, lymphoid, neoplasms, and autoimmune diseases. Some autoantibodies have higher affinity to high molecular weight-VWF multimers (HMW-VWFMs); clearance of HMW-VWFMs leads to bleeding.</p><p>2) Non-immune mechanisms induced reduction of VWF activity</p><p>One of the mechanisms is VWF adsorption onto malignant cells and paraproteins (i.e., as in multiple myeloma) and thereby removed from the blood circulation. VWF-linked proteolysis can be induced by shear stress. According to high levels of shear stress in AS and LVAD, HM-VWFMs are more susceptible to cleavage by ADAMTS13. We will find a large number of AVWS cases related to cardiovascular diseases.</p>

Journal

  • Rinsho Ketsueki

    Rinsho Ketsueki 61 (7), 809-817, 2020

    The Japanese Society of Hematology

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