Laboratory monitoring of replacement therapy for major surgery in von Willebrand disease

P. M. Mannucci, M. Franchini

Research output: Contribution to journalReview articlepeer-review

Abstract

Von Willebrand disease (VWD) is an inherited haemorrhagic disorder caused by a quantitative or qualitative defect of von Willebrand factor (VWF), a multimeric plasma glycoprotein that plays a key role in platelet adhesion to the subendothelium and acts as a carrier of factor VIII (FVIII) in blood. Patients with VWD experience bleeding symptoms that are mainly localized in mucous membranes and soft tissues, and their severity depends on the degree of the primary reduction in VWF and the secondary deficiency of FVIII in plasma. Because VWD patients are also at increased risk of perioperative bleeding, a prophylactic treatment aimed to correct the dual haemostatic defect (i.e. VWF and FVIII) is warranted. This review summarizes knowledge on the current management of patients undergoing major surgery, focusing on the peri-surgical laboratory monitoring of replacement therapy with VWF/FVIII concentrates. We suggest to monitor plasma levels of FVIII coagulant activity in the postoperative period rather than a surrogate maker of platelet-binding VWF activity as the ristocetin cofactor assay and its recent modifications.

Original languageEnglish
Pages (from-to)182-187
Number of pages6
JournalHaemophilia
Volume23
Issue number2
DOIs
Publication statusPublished - Mar 1 2017

Keywords

  • bleeding
  • major surgery
  • von Willebrand disease
  • VWF/FVIII concentrates

ASJC Scopus subject areas

  • Hematology
  • Genetics(clinical)

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