How I treat von Willebrand disease.
Recent multicenter studies have clarified the molecular basis underlying the different von Willebrand disease (VWD) types, all of which are caused by the deficiency and/or abnormality of von Willebrand factor (VWF). These studies have suggested a unifying pathophysiologic concept. The diagnosis of VWD, remains difficult because its clinical and laboratory phenotype is very heterogeneous and may overlap with normal subjects. Stringent criteria are therefore required for a clinically useful diagnosis. In this paper, we delineate a practical approach to the diagnosis and treatment of VWD. Our approach is based on the critical importance of a standardized bleeding history that has been condensed into a final bleeding score and a few widely available laboratory tests, such as VWF ristocetin cofactor activity, VWF antigen and factor VIII. This approach would help identify those subjects who will probably benefit from a diagnosis of VWD. The next step involves performing a trial infusion with desmopressin in all patients who fail to exhibit an enhanced responsiveness to ristocetin. On the basis of these results and through a series of illustrative examples, the clinician will be able to select the best approach for the optimal management of VWD, according to the patient's characteristics and clinical circumstances.
Rodeghiero
Francesco
F
Department of Cell Therapy and Hematology, San Bortolo Hospital, Vicenza, Italy. rodeghiero@hemato.ven.it
Castaman
Giancarlo
G
Tosetto
Alberto
A
D000293:Adolescent; D000328:Adult; D003894:Deamino Arginine Vasopressin / Q000008:administration & dosage*; D005260:Female; D006470:Hemorrhage / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D006490:Hemostatics / Q000008:administration & dosage*; D006801:Humans; D008297:Male; D008875:Middle Aged; D015337:Multicenter Studies as Topic; D012310:Ristocetin / Q000008:administration & dosage*; D014842:von Willebrand Diseases / Q000097:blood* / Q000175:diagnosis* / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000032:analysis
D006490:Hemostatics; D014841:von Willebrand Factor; D012310:Ristocetin; D003894:Deamino Arginine Vasopressin