What causes acquired von Willebrand syndrome?

Updated: Jul 19, 2021
  • Author: Vadim Kostousov, MD; Chief Editor: Eric B Staros, MD  more...
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Due to autoimmune clearance or inhibition, as follows:

  • Lymphoproliferative diseases (lymphoma, leukemia)

  • Monoclonal gammopathies (multiple myeloma, Waldenstrom macroglobulinemia)

  • Systemic lupus erythematosus and other autoimmune disorders

  • Some cancers (Wilms tumor, Ewing sarcoma, carcinoma)

Due to increased shear-induced proteolysis (vWF:Ag is often normal or even elevated), as follows:

  • Ventricular septal defect

  • Aortic stenosis

  • Primary pulmonary hypertension

  • Extracorporeal life support

  • Due to other or unknown mechanisms, as follows:

  • Hypothyrosis

  • Drug-induced (hydroxyethyl starch, valproic acid)

  • Myeloproliferative diseases (polycythemia, thrombocythemia)

  • Angiodysplasia, glycogen storage disease

Increased vWF:Ag level and vWF:RCo activity are observed in acute phase reactions, as follows:

  • Stress and extensive exercise

  • Inflammation

  • Cancer

  • Obesity

  • Postoperative period

  • Diabetes

  • Atherosclerosis and atherothrombosis

  • Pregnancy

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