/von Willebrand disease 3

von Willebrand disease 3

Rare Disease

MONDO:0010191

Also Known As

VWD type 3VWD3von Willebrand disease 3von Willebrand disease type 3von Willebrand disease type IIIvon Willebrand's disease 3von Willebrand's disease type 3VON WILLEBRAND disease, type 3VWD, type 3Von Willebrand disease, type 3

Definition

Type 3 von Willebrand disease (type 3 VWD) is the most severe form of VWD characterized by a bleeding disorder associated with a total or near-total absence of Willebrand factor (von Willebrand factor; VWF) in the plasma and cellular compartments, also leading to a profound deficiency of plasmatic factor VIII (FVIII).