Surgery in Patients with Von Willebrand Disease : A Single Center Experience / 임상소아혈액종양

ABSTRACT

BACKGROUND: Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Surgery, even relatively minor procedures, in patients with moderate to severe qualitative and quantitative deficiencies of von Willebrand factor (VWF) can be associated with a life-threatening risk of excessive bleeding. The purpose of this study was to evaluate the safety and efficacy of VWF/FVIII in patients with von Willebrand disease before surgery and determine the efficacy of VWF/FVIII.METHODS: We reviewed the results of surgical procedures in patients with VWD at Kyung Hee University Hospital at Gangdong, between September 2009 and January 2016. VWF/FVIII concentrates were administrated preoperatively to all patients.RESULTS: Between September 2009 and January 2016 at our center, eight surgical procedures were performed successfully and no severe complications were observed in the seven patients with VWD. Four orthopedic procedures, one laparoscopic appendectomy, one ovary cystectomy, one strabotomy, and one dental extraction were performed. The median duration of hospitalization was seven days. VWF/FVIII concentrates were administered prior to all procedures, including the dental extraction. In all cases, uncontrolled bleeding and thromboembolic complications were not observed.CONCLUSION: Patients with VWD who require surgery can be treated efficiently and safely with VWF/FVIII concentrates. There is excellent tolerance, efficacy and safety in preventing excessive bleeding during surgery. When administering VWF/FVIII concentrates, treatment should be monitored with VWF Ag, VWFRCo and FVIII plasma levels.