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ABSTRACT

von Willebrand disease (VWD) type 3 is a rare disorder characterized by absent or <0.1 UmL(-1) of ristocetin cofactor (VWF:RCo), and a very low level of factor VIII (FVIII:C). A total absence of FVIII:C has never been reported in type 3 VWD. This case illustrates the effect of severe von Willebrand factor (VWF) deficiency on the factor VIII level.

Subject(s)

Diagnostic Errors , Factor VIII/genetics , Hemophilia A/genetics , Postoperative Hemorrhage/genetics , von Willebrand Disease, Type 3/blood , von Willebrand Factor/metabolism , Circumcision, Male/adverse effects , Female , Genes, Recessive , Genotype , Hemophilia A/diagnosis , Hemostatics/therapeutic use , Humans , Infant , Male , Pedigree , Postoperative Hemorrhage/drug therapy , von Willebrand Disease, Type 3/diagnosis , von Willebrand Disease, Type 3/genetics

ABSTRACT

Subject(s)

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