Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Aug;109(2):121-128.
doi: 10.1111/ejh.13785. Epub 2022 May 24.

Use of the von Willebrand factor concentrate with low factor VIII content to manage patients with inherited von Willebrand disease requiring surgical or secondary long-term prophylaxis: An expert opinion paper from an Italian panel

Giancarlo Castaman  1 , Alessandra Borchiellini  2 , Antonio Coppola  3 , Dorina Cultrera  4 , Renato Marino  5 , Augusto B Federici  6 , Anna Chiara Giuffrida  7 , Emanuela Marchesini  8 , Angelo Claudio Molinari  9 , Siboni Simona Maria  10 , Ezio Zanon  11
Affiliations
Review

Use of the von Willebrand factor concentrate with low factor VIII content to manage patients with inherited von Willebrand disease requiring surgical or secondary long-term prophylaxis: An expert opinion paper from an Italian panel

Giancarlo Castaman et al. Eur J Haematol. 2022 Aug.

Abstract

Objectives: The present review aims to summarize the state-of-the-art von Willebrand disease (VWD) treatment focusing on specific clinical settings (obstetrics, surgery, long-term prophylaxis and comorbidities) as well as on the use of a Von Willebrand factor (VWF) concentrate with low FVIII content.

Methods: Literature research and case reports.

Results and conclusions: Considering that patients affected by VWD have an intact ability to synthesize FVIII, in order to avoid excessive levels of FVIII, a highly purified plasma VWF concentrate with low FVIII content could be particularly useful in those patients and clinical circumstances at high thrombotic risk as well as for long-term prophylaxis. When deciding the optimal therapeutic strategy, physicians should take into account both the patient's history and the differences among available concentrates according to the clinical situations requiring treatment.

Keywords: long-term prophylaxis; plasma derived von Willebrand factor; surgical prophylaxis; von Willebrand disease.

PubMed Disclaimer

References

REFERENCES

    1. Peyvandi F, Garagiola I, Baronciani L. Role of von Willebrand factor in the haemostasis. Blood Transfus Trasfus Sangue. 2011;9(suppl 2):s3-s8.
    1. Leebeek FW, Eikenboom JC. Von Willebrand's disease. N Engl J Med. 2016;375(21):2067-2080.
    1. Castaman G, Linari S. Diagnosis and treatment of von Willebrand disease and rare bleeding disorders. J Clin Med. 2017;6(4):45.
    1. Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH ISTH NHF WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021;5(1):301-325.
    1. Borel-Derlon A, Federici AB, Roussel-Robert V, et al. Treatment of severe von Willebrand disease with a high-purity von Willebrand factor concentrate (Wilfactin): a prospective study of 50 patients. J Thromb Haemost. 2007;5:1115-1124.

MeSH terms

Grants and funding

LinkOut - more resources