Current management of von Willebrand's disease.
von Willebrand's disease (vWD) is the most frequent inherited disorder and is the result of a deficiency and/or abnormality of von Willebrand factor (vWF). As a consequence, the level of factor VIII, the presence of which reflects the ability of vWF to stabilise it, is usually low. Bleeding time, which reflects the ability of vWF to promote platelet adhesion to subendothelium, is therefore prolonged. However, from a therapeutic point of view, it appears that the correction of factor VIII and bleeding time is sufficient to prevent or treat bleeding in these patients. There are 2 main therapeutic tools to improve or normalise these major determinants of the bleeding tendency in this disorder. The majority of patients, identified by a test infusion, can be successfully treated by giving desmopressin (DDAVP), a synthetic analogue of vasopressin. Desmopressin is able to induce the increase of autologous vWF released from endothelial cells, leading to the correction of factor VIII levels and of bleeding time. In the remaining cases, blood products, namely factor/vWF concentrates, are required to accomplish haemostasis. All these concentrates are able to correct factor VIII levels, whereas their effect on bleeding time may not be consistent. The modern virucidal techniques abolish the risk of transmission of blood-borne viruses (e.g. hepatitis viruses and HIV) and make these products safer than blood-bank cryoprecipitate.
Castaman
G
G
Department of Haematology and Haemophilia, San Bortolo Hospital, Vicenza, Italy.
Rodeghiero
F
F
D005260:Female; D006801:Humans; D011247:Pregnancy; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology