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Clinical Approach to Type 3 von Willebrand Disease

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Pediatric Bleeding Disorders

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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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Authors and Affiliations

  1. Department of Pediatrics, Division of Pediatric Hematology/Oncology/Stem Cell Transplant, Columbia University Irving Medical Center, Children’s Hospital of New York/New York Presbyterian Morgan Stanley Children’s Hospital, New York, NY, USA

    Dominder Kaur

  2. Nationwide Children’s Hospital, Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Columbus, OH, USA

    Sarah H. O’Brien

  3. Center for Innovation in Pediatric Practice, Abigail Wexner Research Institute at Nationwide Children’s Hospital, Columbus, OH, USA

    Sarah H. O’Brien

  4. Department of Pediatrics, The Ohio State University College of Medicine, Columbus, OH, USA

    Sarah H. O’Brien

Authors
  1. Dominder Kaur
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  2. Sarah H. O’Brien
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Corresponding author

Correspondence to Dominder Kaur .

Editor information

Editors and Affiliations

  1. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Amy L. Dunn

  2. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Bryce A. Kerlin

  3. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Sarah H. O'Brien

  4. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Melissa J. Rose

  5. Division of Pediatric Hematology, Oncology and Bone Marrow Transplant, Nationwide Children’s Hospital, Columbus, OH, USA

    Riten Kumar

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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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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-31660-0

  • Online ISBN: 978-3-030-31661-7

  • eBook Packages: MedicineMedicine (R0)

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