The management of patients with congenital von Willebrand disease during surgery or other invasive procedures: focus on antihemophilic factor/von Willebrand factor complex.
Von Willebrand disease, the most common hereditary bleeding disorder, arises from quantitative or qualitative defect of von Willebrand factor (VWF). The aim of the treatment is to correct the dual defect of hemostasis caused by the abnormal/reduced VWF and the concomitant deficiency of factor VIII (FVIII). The synthetic vasopressin analogue desmopressin is the mainstay of therapy in about 80% of patients, while nearly 20% are unresponsive and must be treated with FVIII/VWF concentrates. This latter therapeutic option will be focused in the review, with particular consideration to the management of surgery and invasive procedures in these patients.
Franchini
Massimo
M
Servizio di Immunoematologia e Trasfusione - Centro Emofilia, Azienda Ospedaliera di Verona.
Lippi
Giuseppe
G
Blood. 2002 Jan 15;99(2):450-6
11781224
J Thromb Haemost. 2007 Jul;5(7):1420-30
17439628
Haemophilia. 2004 Oct;10 Suppl 4:169-76
15479393
Blood. 2001 Apr 1;97(7):1915-9
11264151
Haemophilia. 1998;4 Suppl 3:48-52
10028319
Am J Hematol. 2001 Apr;66(4):280-4
11279640
Thromb Haemost. 1998 Mar;79(3):456-65
9531024
Haemophilia. 2004 May;10(3):199-217
15086318
Haemophilia. 2004 Jan;10(1):42-51
14962219
Crit Rev Clin Lab Sci. 2007;44(2):115-49
17364690
Best Pract Res Clin Haematol. 2001 Jun;14(2):257-79
11686099
Haematologica. 2007 Jul;92(7):944-51
17606445
J Thromb Haemost. 2007 Jun;5(6):1115-24
17403090
Haemophilia. 2002 Sep;8(5):607-21
12199668
Haemophilia. 2003 Nov;9(6):688-95
14750934
Haemophilia. 2001 Jan;7 Suppl 1:10-5
11240613
N Engl J Med. 2000 Aug 17;343(7):457-62
10950667
N Engl J Med. 2004 Aug 12;351(7):683-94
15306670
Blood. 1987 Feb;69(2):454-9
3492222
Haematologica. 2003 Nov;88(11):1279-83
14607757
Haematologica. 2003 Jan;88(1):94-108
12551832
Thromb Haemost. 2002 Feb;87(2):224-30
11858481
Haemophilia. 2002 Nov;8(6):761-7
12410644
Haemophilia. 2000 Jul;6 Suppl 1:60-7
10982270
Haemophilia. 1998;4 Suppl 3:33-9
10028316