Diagnosis of von Willebrand disease

摘要: Summary. von Willebrand disease (vWD) is a bleeding disorder caused by quantitative or qualitative defects of factor (vWF). vWF synthesized endothelial cells and megakaryocytes circulates in plasma as multimeric high molecular weight glycoprotein. plays major role the early phases ostasis promoting platelet-vessel wall plateletplatelet interactions under shear conditions. It also carrier coagulation VIII (FVIII) plasma. A deficiency results impairment both primary secondary ostasis. Therefore, patients with vWD manifest symptoms that are typical (mucocutaneous haemorrhages) but, case severe vWF, there haemarthroses haematomas, which those seen defects. Several types subtypes have been described degree heterogeneity. The diagnosis based on measurements platelet ability to interact its receptor analysis composition vWF. Due heterogeneity defects, correct may be sometimes difficult but very important for an appropriate treatment vWD.