الوصف
von Willebrand disease (vWD) is a common autosomal human bleeding disorder caused by the defective synthesis or function of the multimeric plasma glycoprotein von Willebrand factor (vWF). vWF is required for platelet adhesion at the site of vascular damage and serves as the carrier and stabiliser for coagulation factor VIII (FVIII) by preventing FVIII proteolytic degradation and inactivation by activated protein C and factor Xa. As a consequence of the protective function of vWF, the level of FVIII in plasma generally coincides with the vWF:Ag level. vWF is synthesised by vascular endothelial cells and megakaryocytes and circulates in blood as a series of multimers containing a variable
number of homodimers.
دواعي الإستعمال
Several types of von Willebrand disease exist; type 1, type 2A, 2B, 2M, 2N and type 3. For correct classification, tests should include bleeding time, Factor VIII activity, vWF antigen (vWF:Ag), vWF activity and vWF multimers.
نوع العينة والكمية والشروط
1 ml Citrated Plasma Frozen
إحتياطات خاصة
von Willebrand & Factor VIII:C are the components of factor VIII.
المعدل الطبيعي
60-150 Titer
