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Semin Thromb Hemost 2017; 43(03): 338-354
DOI: 10.1055/s-0036-1585080
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Monitoring Therapy during Treatment of von Willebrand Disease

Emmanuel J. Favaloro
1   Sydney Centres for Thrombosis and Haemostasis, Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Westmead, New South Wales Australia
2   Pathology West, NSW Health Pathology, New South Wales, Australia
,
Leonardo Pasalic
1   Sydney Centres for Thrombosis and Haemostasis, Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Westmead, New South Wales Australia
2   Pathology West, NSW Health Pathology, New South Wales, Australia
,
Jennifer Curnow
1   Sydney Centres for Thrombosis and Haemostasis, Departments of Clinical and Laboratory Haematology, Institute of Clinical Pathology and Medical Research and Westmead Hospital, Westmead, New South Wales Australia
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Publikationsdatum:
29. Juli 2016 (online)

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Abstract

von Willebrand factor (VWF) is an adhesive plasma protein that primarily acts to bridge platelets to sites of vascular injury and thus prevent bleeding. von Willebrand disease (VWD) is the most common inherited bleeding disorder and is caused by deficiency and/or defects of VWF, leading to low levels of plasma VWF or dysfunctional VWF. Factor VIII (FVIII) is also reduced in many patients with VWD, since VWF stabilizes and protects FVIII from degradation. Treatment of VWD primarily entails replacement of VWF, and sometimes FVIII, to protect against bleeding. This may entail use of VWF/FVIII concentrates, and/or desmopressin (1-deamino-8-d-arginine vasopressin) to release endogenous VWF in some patients. Adjunct therapies include antifibrinolytics and hormonal therapies in women. Optimal treatment of VWD entails measuring the effects of treatment, either as a trial before surgery or during therapeutic management. This is usually accomplished by performance of the same tests that are used to help diagnose VWD, although additional monitoring (clinically and/or by laboratory testing) may also be performed. The current review provides an overview of the treatment of VWD but is primarily focused on the monitoring of such therapy.

Keywords

von Willebrand disease - VWD - diagnosis - management - factor concentrate - therapy - monitoring