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von Willebrand Disease

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Congenital Bleeding Disorders

Abstract

von Willebrand disease (VWD) is the most common congenital bleeding disorder with an estimated incidence of 1% in the general population. The disorder is classified into three main types: type 1 and type 3 as quantitative deficiency and type 2 as qualitative defects. The bleeding tendency is highly variable in VWD, ranging from an asymptomatic condition, mainly in type 1 VWD, to severe life-threatening hemorrhage, most notably in type 3 VWD. Diagnosis of the disorder is sophisticated and requires advanced laboratory testing and precise clinical assessment. Bleeding symptoms, decreased VWF level and/or activity, and the pattern of inheritance are three main criteria for appropriate and timely diagnosis of VWD. Although on-demand therapy is the mainstay of treatment in VWD, long-term prophylaxis is recommended for those with recurrent and severe hemorrhages. Different therapeutic choices are available in VWD including VWF/FVIII concentrates, recombinant VWF, desmopressin, and, in some locations, fresh frozen plasma (FFP). With timely diagnosis and appropriate management of the VWD, the burden of the disorder can be alleviated or even eliminated.

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Authors and Affiliations

  1. Department of Hematology and Blood Transfusion, School of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran

    Akbar Dorgalaleh, Shadi Tabibian & Yavar Shiravand

  2. Haematology, Institute of Clinical Pathology and Medical Research, NSW Health Pathology, Westmead Hospital, Westmead, NSW, Australia

    Emmanuel J. Favaloro

  3. Sydney Centres for Thrombosis and Haemostasis, Westmead, NSW, Australia

    Emmanuel J. Favaloro

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  1. Akbar Dorgalaleh
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  2. Shadi Tabibian
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  3. Yavar Shiravand
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  4. Emmanuel J. Favaloro
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Correspondence to Emmanuel J. Favaloro .

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Editors and Affiliations

  1. Iran University of Medical Sciences, Departement of Hematology and Blood TransfusionSchool of Allied Medicine, Iran University of Medical Sciences, Tehran, Iran

    Akbar Dorgalaleh

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Dorgalaleh, A., Tabibian, S., Shiravand, Y., Favaloro, E.J. (2018). von Willebrand Disease. In: Dorgalaleh, A. (eds) Congenital Bleeding Disorders . Springer, Cham. https://doi.org/10.1007/978-3-319-76723-9_3

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  • DOI: https://doi.org/10.1007/978-3-319-76723-9_3

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