Table of Contents
To view the fulltext, log-in or choose pay-per-view options:
Get Access

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

Sucker C.a · Michiels J.J.c · Zotz R.B.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 Acta Haematol 2009;121:177–182 (DOI:10.1159/000214858)

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.

 

Individual Users: Register with Karger Login Information

Please create your User ID & Password





Contact Information











I have read the Karger Terms and Conditions and agree.


Pay-per-View Options
Direct payment This item at the regular price: USD 38.00
Payment from account With a Karger Pay-per-View account (down payment USD 150) you profit from a special rate for this and other single items.
This item at the discounted price: USD 26.50