von Willebrand disease

Akbar Dorgalaleh, Shadi Tabibian, Yavar Shiravand, Emmanuel J. Favaloro

Research output: Book chapter/Published conference paperChapter (peer-reviewed)peer-review

3 Citations (Scopus)

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.
Original languageEnglish
Title of host publicationCongenital bleeding disorders
Subtitle of host publicationDiagnosis and management
EditorsAkbar Dorgalaleh
Place of PublicationCham, Switzerland
PublisherSpringer
Chapter3
Pages57-102
Number of pages46
ISBN (Electronic)9783319767239
ISBN (Print)9783319767222
DOIs
Publication statusE-pub ahead of print - 26 Jul 2018

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