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How I treat von Willebrand disease Rodeghiero F; Castaman G; Tosetto ABlood 2009[Aug]; 114 (6): 1158-65Recent 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.|Adolescent[MESH]|Adult[MESH]|Deamino Arginine Vasopressin/*administration & dosage[MESH]|Female[MESH]|Hemorrhage/blood/diagnosis/drug therapy[MESH]|Hemostatics/*administration & dosage[MESH]|Humans[MESH]|Male[MESH]|Middle Aged[MESH]|Multicenter Studies as Topic[MESH]|Ristocetin/*administration & dosage[MESH]|von Willebrand Diseases/*blood/*diagnosis/*drug therapy[MESH]|von Willebrand Factor/analysis[MESH] |
