The mother of a 7-year-old girl was diagnosed recently as having von Willebrand factor disease. The girl has had two prolonged nosebleeds this winter, and her mother requests testing. What tests can be used to screen for this condition?

von Willebrand factor disease (vWD) is the most common inherited bleeding disorder. It typically is transmitted in an autosomal dominant fashion and has an estimated prevalence in the United States of 1%. The disorder results from a decreased quantity or activity of von Willebrand factor (vWF), a protein essential for primary platelet hemostasis and fibrin clot formation. Clinical manifestations range from an asymptomatic state to mild disease with epistaxis, easy bruising, or menorrhagia to severe disease with life-threatening bleeding.

Screening patients for vWD is difficult because of the disease’s heterogeneity, multiple subtypes, and broad spectrum of severity. In addition, there is a wide physiologic range of vWF concentrations, which do not...

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