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

Abstract number: P0608

Smaradottir A., Bona R., Fritts L.

University of Connecticut Health Center, USA

Acquired von Willebrand syndrome (AvWS) is a rare bleeding disorder that usually occurs in elderly patients with no previous personal or family history of a bleeding disorder. Various underlying diseases have been associated with AvWS, most commonly hematoproliferative disorders, but also solid tumors, various autoimmune disorders and drugs. We present a case of a 63-year-old man with AvWS who needed thyriodectomy. He was treated successfully with recombinant Factor VIIa (rFVIIa) perioperatively, after trials with conventional therapies had failed. The patient, who previously had no history of bleeding diathesis, sustained a major soft tissue hemorrhage of his upper back after trauma. During work up his von Willebrand activity was found to be 0 and von Willebrand multimer analysis showed deficiency of intermediate and high molecular weight multimers. Factor VIII level was 7%. On evaluation he was also found to have a monoclonal gammopathy of unknown significance and cold nodules in the thyroid gland. Because of his coagulopathy, it was considered unsafe to do a fine needle biopsy and the decision was made to do a thyroidectomy. This patient had previously been treated with DDAVP, IVIG, steroids and Humate-P with no or minimal response. Because of a high risk of bleeding it was decided to treat him with rFVIIa, 90 micrograms/kg perioperatively. Patient received one dose 30 min prior to surgery and a total of two doses intraoperatively. No significant bleeding occurred and adequate clot formation was observed during surgery. Postoperatively, the patient was treated with rFVIIa every 2–3 h for approximately 72 h, total number of doses were 32. Hospital course was without complications apart from superficial thrombophlebitis which developed at the infusion site of rFVIIa after 25 doses. In summary our patient was successfully treated with rFVIIa during neck surgery when other treatment trials had been unsuccessful. In vitro studies have shown that rFVIIa can activate coagulation at activated platelet surfaces, which can lead to a stable fibrin clot, despite lack of initial platelet plug in patients with defects in primary hemostasis. We conclude that rFVIIa can be considered an alternative treatment option in patients with AvWS who do not respond to other therapies.