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DOI: 10.5482/ha-1129
Erworbenes von-Willebrand-Syndrom Typ 2A als Begleiterscheinung eines Dialyseshunts?
Acquired von Willebrand’s disease type 2A following arteriovenous fistula for haemodialysis?Publication History
09 December 2010
Publication Date:
28 December 2017 (online)
Summary
Acquired von Willebrand’s disease (aVWD) is considered to be an underestimated cause of unexplained bleeding. Adsorption of von Willebrand factor (VWF) to tumour cells or hydroxyethyl starch and elimination of VWF by autoantibodies as well as shear stress-induced mechanical alteration of VWF with concomitant cleavage by enzymes may lead to an acquired deficiency of VWF and a bleeding disorder.
We report a 39-year-old woman who developed spontaneous bleeding five years after surgical creation of an arteriovenous fistula (AVF) for haemodialysis treatment. AVWD type 2A was diagnosed after successful renal transplantation. One year after surgical closure of the AVF, the aVWD could not be verified again. Thus, the aVWD may have developed because of altered blood flow and shear stress inside the arteriovenous fistula.
Zusammenfassung
Das erworbene von-Willebrand-Syndrom (VWS) gilt als unterschätzte Ursache vermeintlich unerklärlicher Blutungsneigungen. Neben der Adsorption des von-Willebrand-Faktors (VWF) an Tumorzellen oder Hydroxyethylstärke sowie der Elimination von VWF durch Autoantikörper kann eine Scherkraft-induzierte mechanische Alterierung des VWF mit nachfolgender enzymatischer Spaltung zu einem erworbenen VWF-Mangel und einer entsprechenden Blutungsneigung führen.
Wir berichten über eine 39-jährige ehemals dialysepflichtige Patientin, bei der es fünf Jahre nach Anlage eines Dialyseshunts zu spontanen Blutungen kam. Die Diagnose eines erworbenen VWS Typ 2A wurde nach erfolgreicher Nierentransplantation gestellt. Ein Jahr nach operativem Shuntverschluss war das VWS nicht mehr nachweisbar. Es kann somit vermutet werden, dass das VWS durch veränderte Strömungsverhältnisse und Scherkräfte im Bereich des Dialyseshunts entstanden war.
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