Preferred Label : Von Willebrand disease type 3;
ICD-11 definition : Type 3 von Willebrand disease 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). The type 3 disease is the rarest form of VWD, accounting
for less than 5% of all cases.;
Origin ID : 805917536;
Automatic exact mappings (from CISMeF team)
Type 3 von Willebrand disease 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). The type 3 disease is the rarest form of VWD, accounting
for less than 5% of all cases.
