A case of acquired von Willebrand syndrome successfully treated with recombinant Factor VIIa during thyroidectomy

Introduction Acquired von Willebrand syndrome (avWs) is a rare bleeding disorder. Affected individuals usually present later in life with skin or mucosal bleeding. This syndrome is often associated with other disorders. These include lymphoproliferative diseases, myelo-proliferative disorders, hypothyroidism and aortic stenosis. In avWs the von Willebrand factor is synthezised normally but high molecular weight multimers seem to be cleared rapidly by mechanisms that are not fully understood. Other pathophysiologic mechanisms implicated in avWS include antibody-antigen complex formation, absorption onto malignant cells or proteolytic degradation of the protein. . . .