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A hereditary family of blood-clotting disorders caused by a deficiency of the von Willebrand factor protein and factor VIII protein, and characterized by prolonged bleeding.

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Pseudohemophilia; Angiohemophilia; Constitutional Thrombopathy; Minot-Von Willebrand Disease; Vascular Hemophilia; Willebrand-Jüergens Disease.

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Genetic, although a rare, acquired form exists.

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Prevalence worldwide is estimated at 1%. Acquired forms are much more rare.

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Autosomal dominant or recessive inheritance has been suggested as potential genetic transmission mode.

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von Willebrand disease is the most common inherited bleeding disorder in humans, and is secondary to abnormalities of von Willebrand factor (vWF). There are several subtypes.

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Mucocutaneous bleeding with normal platelet count, elevated bleeding time, decreased ristocetin cofactor activity (ristocetin is an antibiotic that alters normal vWF structure, causing platelet aggregation), decreased vWF antigen (can be normal in type II), decreased factor VIII activity; gel electrophoresis to determine vWF structure.

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There are several subtypes. Type I has a partial quantitative defect with normal structure, comprises 70% of cases, and has mild to moderate bleeding; it is autosomal dominant. Type II has both qualitative and quantitative defects. Type IIA (10% of cases) has mild to moderate bleeding and a poor response to desmopressin acetate, while type IIB (<5% of cases) has mild to moderate bleeding, thrombocytopenia, and a contraindication to desmopressin acetate. Type IIM (reportable) has a variable bleeding disorder with decreased vWF:Ag, vWF activity, and factor VII:C. Type IIN (reportable and autosomal recessive) has a variable bleeding disorder that may resemble hemophilia A. Type III (severe and rare, may be autosomal recessive) has a complete deficiency of vWF. Acquired von Willebrand Disease is associated with myeloproliferative disease, hypothyroidism, B-cell disorders, and cardiovascular defects, resembles type 1 or 2, and has decreased plasma vWF antigen and normal platelet vWF antigen.

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Treatment:Desmopressin acetate, which increases the release of vWF, has a favorable response in 80% of type I patients. The response in type II diseases is variable, because an increased release of qualitatively poor vWF is not useful for platelet binding. Desmopressin acetate may be contraindicated in type IIB, because platelet aggregation may exacerbate thrombocytopenia, but this is controversial. Estrogen may also increase production of vWF.

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Replacement therapy, for failures or contraindications to desmopressin acetate, was formerly with cryoprecipitate. However, virally inactivated concentrates of factor VIII now exist, although the content of vWF varies.

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The products of choice are Hemate-P and VHP vWF concentrate. Treatment is empiric, although it should be continued for 7 to 10 days after major surgical procedures, and for 3 to 5 days after minor ones; postpartum hemorrhage may require 1 month of therapy. A suggested regimen for Hemate-P in type III von Willebrand disease for major surgery is bolus of ...

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