Title
Acquired von Willebrand syndrome : experience from 2 years in a single laboratory compared with data from the literature and an International Registry Acquired von Willebrand syndrome : experience from 2 years in a single laboratory compared with data from the literature and an International Registry
Author
Faculty/Department
University Hospital Antwerp
Publication type
article
Publication
New York ,
Subject
Human medicine
Source (journal)
Seminars in thrombosis and hemostasis. - New York
Volume/pages
28(2002) :2 , p. 227-237
ISSN
0094-6176
ISI
000175384200012
Carrier
E
Target language
English (eng)
Full text (Publishers DOI)
Affiliation
University of Antwerp
Abstract
Acquired von Willebrand syndrome (avWS) has gained more attention during the last years. An International Registry has been compared with the literature. It could be shown that the data collected from 123 publications compared well with the data from the registry, albeit with differences in the number of patients suffering from lymphoproliferative diseases and cardiovascular disorders, that were more prominent in the registry and the group of miscellaneous conditions underrepresented in the registry. Our data are clearly different for the lymphoproliferative diseases with only four patients in 2 years. These patients usually suffer from severe bleeding complications together with low to very low factor VIII/von Willebrand factor (FVIII/vWF) concentrations and thus will not go undiagnosed. In contrast to this, patients with cardiovascular disorders usually bleed only during surgical procedures or catheter procedures. At that time they have increased vWF parameters. Because of this and because the acute bleeding is of limited duration and usually not life threatening, many of them are discharged without a proper diagnosis and are only rarely referred to a specialized diagnostic work-up thereafter. In conclusion, avWS, although not a frequent disease, is nevertheless clearly, underdiagnosed. This should be addressed in future prospective studies.
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