Abstract
Type 3 von Willebrand disease (VWD) is a severe quantitative deficiency of von Willebrand factor (VWF) that results in negligible circulating factor levels. As VWF is the carrier protein for factor VIII (FVIII), there is an accompanying FVIII deficiency. Patients with type 3 VWD experience more frequent bleeding symptoms than other VW types. Spontaneous internal bleeding, like joint and muscle bleeding, can also occur. Diagnosis is made by quantifying VW antigen and activity levels, which are typically <10%. Factor replacement is the mainstay of therapy, and this is for both VWF and FVIII. Adjunctive therapies are limited to antifibrinolytic agents, as desmopressin is not helpful in type 3 VWD.
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Kaur, D., O’Brien, S.H. (2020). Clinical Approach to Type 3 von Willebrand Disease. In: Dunn, A., Kerlin, B., O'Brien, S., Rose, M., Kumar, R. (eds) Pediatric Bleeding Disorders. Springer, Cham. https://doi.org/10.1007/978-3-030-31661-7_11
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DOI: https://doi.org/10.1007/978-3-030-31661-7_11
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