TABLE I.

Disorders Pathophysiologically Associated with Acquired von Willebrand Syndrome (AVWS)

Pathophysiologic category	Disease or association
Antibodies to VWF	Lymphoproliferative diseases, monoclonal gammopathies, or autoimmune diseases such as systemic lupus erythematosus
Shear-induced VWF conformational changes leading to increased proteolysis of VWF	Ventricular septal defect, aortic valvular stenosis, hypertrophic obstructive cardiomyopathy, left ventricular assist device, or primary pulmonary hypertension
Markedly elevated blood platelet count	Essential thrombocythemia, polycythemia vera, myeloid metaplasia with myelofibrosis, or other myeloproliferative disorders
Removal of VWF from circulation by aberrant binding to tumor cells	Wilm’s tumor and certain lymphoproliferative or plasma cell proliferative disorders
Decreased VWF synthesis	Hypothyroidism
Drugs associated with AVWS	Ciprofloxacin, valproic acid, hydroxyethyl starch, and griseofulvin

AVWS, acquired von Willebrand syndrome; VWF, von Willebrand factor.