[Diagnosis and management of von Willebrand disease]

von Willebrand disease (VWD) is an inherited bleeding disorder resulting from either a quantitative or a qualitative deficiency in the plasma glycoprotein von Willebrand factor (VWF). A diagnosis of VWD can be made when a patient presents with appropriate bleeding and VWF <30 IU/dl. However, persons with VWF levels of 30-50 IU/dl cannot be precluded from the diagnosis of VWD. Desmopressin acetate (DDAVP) or VWF-containing factor VIII (pdVWF/FVIII) concentrate is used for treating VWD. The effect of DDAVP varies among individuals; a trial should be performed while in a nonbleeding state. For patients in whom DDAVP is invalid or those that require long-term management of hemostasis, pdVWF/FVIII is administered. The treatment of the hype rmenorrhea is the hope of every pregnant patient with VWD. When a patient with VWD becomes pregnant, VWF and FVIII should be regularly monitored throughout pregnancy. During childbirth, pdVWF/FVIII concentrate should be administered to achieve VWF and FVIII levels of ≥50 IU/dl before delivery.