Management of inherited von Willebrand disease in 2006.

The aim of treatment of von Willebrand disease (VWD) is to correct the dual defect of hemostasis (i.e., the abnormal platelet adhesion due to reduced and/or dysfunctional von Willebrand factor [VWF] and the abnormal coagulation expressed by low levels of factor [F] VIII). Desmopressin acetate (DDAVP) is the treatment of choice for type 1 VWD because it can induce release of normal VWF from cellular compartments. Prospective studies on biol. response vs. clin. efficacy of DDAVP in VWD types 1 and 2 are in progress to explore its benefits and limits as a therapeutic option. In type 3 and in severe forms of type 1 and 2 VWD, DDAVP is not effective, and for these patients plasma virally inactivated concs. contg. VWF and FVIII are the mainstay of treatment. Several intermediate- and high-purity VWF/FVIII concs. are available and have been shown to be effective in clin. practice (bleeding and surgery). New VWF products almost devoid of FVIII are now under evaluation in clin. practice. Although thrombotic events are rare in VWD patients receiving repeated infusions of concs., there is some concern that sustained high FVIII levels may increase risk of postoperative venous thromboembolism. Dosage and timing of VWF/FVIII administrations should be planned to keep the FVIII level between 50 and 150 U/dL. Appropriate dosage and timing in repeated infusions are also very important in patients exposed to secondary long-term prophylaxis for recurrent bleedings. [on SciFinder (R)]