단일기관에서 시행한 폰빌레브란트병 환자의 수술 경험 = Surgery in Patients with Von Willebrand Disease : A Single Center Experience

다국어 초록 (Multilingual 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, VWF:RCo and FVIII plasma levels.

참고문헌 (Reference)

1 이명숙, "가능성 있는 폰빌레브란트병의 유병률에 대한 연구" 대한소아혈액종양학회 21 (21): 16-22, 2014

2 Mannucci PM, "Treatment of von Willebrand's disease" 351 : 683-694, 2004

3 Federici AB, "The use of desmopressin in von Willebrand disease: the experience of the first 30 years (1977-2007)" 14 (14): 5-14, 2008

4 The Korean Society of Pediatric Hematology Oncology, "The clinical manual of hemophilia and other congenital coagulopathy"

5 Franchini M, "Surgical prophylaxis in von Willebrand's disease: a difficult balance to manage" 6 (6): s33-s38, 2008

6 Zulfikar B, "Surgery in patients with von Willebrand disease" 27 : 812-816, 2016

7 Thompson AR, "Successful treatment for patients with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P)" 10 : 42-51, 2004

8 Castaman G, "Response to desmopressin is influenced by the genotype and phenotype in type 1 von Willebrand disease (VWD): results from the European Study MCMDM-1VWD" 111 : 3531-3539, 2008

9 "Report on the annual global survey 2014" World Federation of Hemophilia

10 Mannuccio Mannucci P, "Prophylactic efficacy and pharmacokinetically guided dosing of a von Willebrand factor/factor VIII concentrate in adults and children with von Willebrand's disease undergoing elective surgery: a pooled and comparative analysis of data from USA and European Union clinical trials" 11 : 533-540, 2013

11 Siboni SM, "Orthopaedic surgery in patients with von Willebrand disease" 20 : 133-140, 2014

12 "Immunate Package leaflet: Information for the user" Baxter AG

13 Mannucci PM, "Evidence-based recommendations on the treatment of von Willebrand disease in Italy" 7 : 117-126, 2009

14 Singleton T, "Emergency department care for patients with hemophilia and von Willebrand disease" 39 : 158-165, 2010

15 Castaman G, "Efficacy and safety during formulation switch of a pasteurized VWF/FVIII concentrate: results from an Italian prospective observational study in patients with von Willebrand disease" 19 : 82-88, 2013

16 Federici AB, "Biologic response to desmopressin in patients with severe type 1 and type 2 von Willebrand disease: results of a multicenter European study" 103 : 2032-2038, 2004

17 Korea Hemophilia Foundation, "2015 Annual report" Korea Hemophilia Foundation 2016