Acquired von Willebrand disease: potential contribution of the VWF:CB to the identification of functionally inhibiting auto-antibodies to von Willebrand factor.

摘要

Acquired von Willebrand disease (AVWD; or syndrome, AVWS) is acknowledged as an 'under-recognized and misdiagnosed' bleeding disorder . The actual incidence of the disorder is unknown, although a recent estimate has suggested an incidence of between 0.04% and 0.15% of the general population and about 4% of patients with VWD . The potential reasons for under-, and/or mis-, diagnosis, probably include combinations of poor recognition by some clinicians and problems with laboratory tests used in the identification of the disorder. For AVWD with a putative auto-immune basis, tests classically involve either mixing studies of patient plasma with normal plasma in a Bethesda-like assay involving various VWF assays, or else direct assessment with an ELISA assay to evaluate the possibility of antibodies against von Willebrand factor (VWF). There are problems with both approaches.