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Von Willebrand disease (vWD) is one of the most common, yet most complex, inherited bleeding disorders. A recent review identified advances in vWD diagnosis and treatment, including the following:
85% of patients have type 1 vWD, a partial deficiency of von Willebrand factor (vWF); the rare type 3 has complete deficiency of vWF.
Patients with type 2 have a qualitative defect in vWF; type 2A patients have defective multimerization, type 2B patients have spontaneous platelet binding, type 2M patients have defective ligand binding, and type 2N patients have defective binding of factor VIII.
Type 2A is often complicated by bleeding from gastrointestinal angiodysplasia.
Bleeding assessment tools, available on the Web, can identify individuals who re…