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Blood, 1 April 2001, Vol. 97, No. 7, pp. 1915-1919
HOW I TREAT
How I treat patients with von Willebrand disease
Pier Mannuccio Mannucci
From the Angelo Bianchi Bonomi Hemophilia and
Thrombosis Center and the Department of Internal Medicine, IRCCS
Maggiore Hospital, University of Milan, Italy.
Von Willebrand disease (vWD) is a frequent inherited disorder of
hemostasis that affects both sexes. Two abnormalities are characteristic of the disease, which is caused by a deficiency or a
defect in the multimeric glycoprotein called von Willebrand factor: low
platelet adhesion to injured blood vessels and defective intrinsic
coagulation owing to low plasma levels of factor VIII. There are 2 main
options available for the treatment of spontaneous bleeding episodes
and for bleeding prophylaxis: desmopressin and transfusional therapy
with plasma products. Desmopressin is the treatment of choice for most
patients with type 1 vWD, who account for approximately 70% to 80% of
cases. This nontransfusional hemostatic agent raises endogenous factor
VIII and von Willebrand factor 3 to 5 times and thereby corrects both
the intrinsic coagulation and the primary hemostasis defects. In
patients with the more severe type 3 and in most patients with type 2 disease, desmopressin is ineffective or is contraindicated and it is
usually necessary to resort to plasma concentrates containing both
factor VIII and von Willebrand factor. Concentrates treated with
virucidal methods should be preferred to cryoprecipitate because they
are equally effective and are perceived as safer.
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