NCIt definition : The most severe form of von Willebrand disease, inherited in an autosomal recessive
pattern. It is characterized by marked deficiency or absence of von Willebrand factor
in the plasma and platelets, and low plasma levels of factor VIII. It may cause severe
bleeding.;
Alternative definition : NICHD: The most severe form of von Willebrand disease, it is inherited in an autosomal
recessive pattern, and is characterized by marked deficiency or absence of von Willebrand
factor in the plasma and platelets, and low plasma levels of factor VIII; it may cause
severe bleeding.;