What is the pathophysiology of type 1 pediatric von Willebrand disease (VWD)?

Updated: Feb 05, 2019
  • Author: Suchitra S Acharya, MD, MBBS; Chief Editor: Hassan M Yaish, MD  more...
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Answer

von Willebrand disease can be classified into 3 main types.

Type 1 von Willebrand disease

Type 1 von Willebrand disease, which accounts for 70-80% of cases, is characterized by a partial quantitative decrease of qualitatively normal von Willebrand factor and FVIII. An individual with type 1 von Willebrand disease generally has mild clinical symptoms, and this type is usually inherited as an autosomal dominant trait; however, penetrance may widely vary in a single family. In addition, clinical and laboratory findings may vary in the same patient on different occasions. Typically, a proportional reduction in von Willebrand factor activity, von Willebrand factor antigen, and FVIII is observed in type 1 von Willebrand disease. [4]


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