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Table 20-8

von Willebrand Disease Classificationa

Type 1Type 2Type 3
Mode of inheritanceADAD or ARAR
Percentage of cases75Approximately 25Rare
PathophysiologyPartial deficiency of vWFQualitatively abnormal vWFMarkedly decreased or absent vWF
Clinical presentationAsymptomatic to severe bleedingModerate to severe bleedingSevere bleeding

Abbreviations: AD, autosomal dominant. AR, autosomal recessive; vWF, von Willebrand factor.


aAdapted from American College of Obstetricians and Gynecologists Committee on Adolescent Health Care. ACOG Committee Opinion No. 580: von Willebrand disease in women. Obstet Gynecol. 2013;122:1368-1373. (Reaffirmed 2017)