Abstract
Most patients with congenital von Willebrand disease (VWD) undergoing major surgical procedures require prophylactic replacement therapy with von Willebrand factor (VWF)/factor VIII (FVIII) concentrates. Venous thromboembolism has been reported in such patients, as a result of a combination of various treatments (i.e., type of surgery, progressive increase of post-infusion FVIII plasma levels) and/or patient-related thrombotic risk factors. On the whole, the literature data show that venous thromboembolic complications in surgical VWD patients who have been prophylactically treated with VWF/FVIII concentrates are extremely rare. Indeed, only 11 cases have been reported in the literature, mostly occurring during orthopedic procedures. Thus, in absence of a widely accepted consensus and adequate prospective studies, we advise that the need for thromboprophylaxis in such patients should be evaluated individually, after a careful risk/benefit analysis.
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References
Rodeghiero F, Castaman G, Dini E (1987) Epidemiological investigation of the prevalence of von Willebrand’s disease. Blood 69:454–459
Castaman G, Federici AB, Rodeghiero F, Mannucci PM (2003) von Willebrand’s disease in the year 2003: towards the complete identification of gene defects for correct diagnosis and treatment. Haematologica 88:94–108
Franchini M, Lippi G (2007) The role of von Willebrand factor in hemorrhagic and thrombotic disorders. Crit Rev Clin Lab Sci 44:115–149. doi:10.1080/10408360600966753
Mannucci PM (2004) Treatment of von Willebrand’s disease. N Engl J Med 351:683–694. doi:10.1056/NEJMra040403
Rodeghiero F, Castaman G (2005) Treatment of von Willebrand disease. Semin Hematol 42:29–35. doi:10.1053/j.seminhematol.2004.10.001
Koster T, Blann AD, Briet E, Vandenbroucke JP, Rosendaal FR (1995) Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 345:152–155. doi:10.1016/S0140-6736(95)90166-3
Makris M, Colvin B, Gupta V, Shields ML, Smith MP (2002) Venous thrombosis following the use of intermediate purity FVIII concentrate to treat patients with von Willebrand’s disease. Thromb Haemost 88:387–388
Mannucci PM (2002) Venous thromboembolism in von Willebrand disease. Thromb Haemost 88:378–379
Mannucci PM, Chediak J, Hanna W, Byrnes J, Marlies L, Ewenstein BM, Alphanate Study Group (2002) Treatment of von Willebrand disease with a high-purity factor VIII/von Willebrand factor concentrate: a prospective, multicenter study. Blood 99:450–456. doi:10.1182/blood.V99.2.450
Lethagen S, Kyrle PA, Castaman G, Haertel S, Mannucci PM, The Haemate P Surgical Study Group (2007) von Willebrand factor/factor VIII concentrate (Haemate P) dosing based on pharmacokinetics: a prospective multicenter trial in elective surgery. J Thromb Haemost 5:1420–1430. doi:10.1111/j.1538-7836.2007.02588.x
Kyrle PA, Minar E, Hirschl M et al (2000) High plasma levels of factor VIII and the risk of recurrent venous thromboembolism. N Engl J Med 343:457–462. doi:10.1056/NEJM200008173430702
Wells PS, Langlois NJ, Webster MA, Jaffey J, Anderson JA (2005) Elevated factor VIII is a risk factor for idiopathic venous thromboembolism in Canada—is it necessary to define a new upper reference range for factor VIII? Thromb Haemost 93:842–846
Mannucci PM, Tenconi PM, Castaman G, Rodeghiero F (1992) Comparison of four virus-inactivated plasma concentrates for treatment of severe von Willebrand disease: a cross-over randomized trial. Blood 79:3130–3137
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Franchini, M., Targher, G., Montagnana, M. et al. Antithrombotic prophylaxis in patients with von Willebrand disease undergoing major surgery: when is it necessary?. J Thromb Thrombolysis 28, 215–219 (2009). https://doi.org/10.1007/s11239-008-0253-7
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DOI: https://doi.org/10.1007/s11239-008-0253-7