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Vol. 121, No. 2-3, 2009
Issue release date: June 2009
Section title: Paper
Acta Haematol 2009;121:177–182
(DOI:10.1159/000214858)

Causes, Etiology and Diagnosis of Acquired von Willebrand Disease: A Prospective Diagnostic Workup to Establish the Most Effective Therapeutic Strategies

Sucker C. · Michiels J.J. · Zotz R.B.
aLaboMed Coagulation Center, Berlin, and bHemostasis Institute Düsseldorf, Düsseldorf, Germany; cHemostasis and Thrombosis Research Center, Antwerp University Hospital, Edegem, Belgium, and Hemostasis Thrombosis Science Center, Goodheart Institute, Rotterdam, The Netherlands

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Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 6/8/2009

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA

Abstract

Acquired von Willebrand disease (aVWD) occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative and myeloproliferative disorders, other malignancies, and cardiovascular disease. aVWD is a complex and heterogeneous defect with a multifactorial etiology and the pathophysiologic mechanisms remain unclear in many cases. Assays for anti-factor VIII (FVIII)/von Willebrand factor (VWF) activities often yield negative results although antibodies may be present in autoimmune disease and some lymphoproliferative disorders. Functional assays of VWF in patients’ plasma and particularly in heart valve disease, VWF multimer analysis are important for aVWD diagnosis. In patients with normal partial thromboplastin times and normal VWF activity, the diagnosis of aVWD is based on clinical suspicion and a careful bleeding history, which should prompt the clinician to initiate further laboratory investigations. Management of bleeding in aVWD relies mainly on desmopressin, FVIII/VWF concentrates and high-dose intravenous immunoglobulin. The half-life of VWF may be very short, and in bleeding episodes high doses of FVIII/VWF concentrates at short intervals may be necessary even when high-dose intravenous immunoglobulin was applied before. Since the optimal treatment strategy has not yet been defined for aVWD of different etiology, controlled multicenter trials aiming at the development of standardized treatment protocols are urgently needed.


Article / Publication Details

First-Page Preview
Abstract of Paper

Published online: 6/8/2009

Number of Print Pages: 6
Number of Figures: 3
Number of Tables: 0

ISSN: 0001-5792 (Print)
eISSN: 1421-9662 (Online)

For additional information: http://www.karger.com/AHA


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Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in goverment regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
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