Acquired von Willebrand syndrome: diagnostic problems and therapeutic options.

Abstract:

:We present a case of acquired von Willebrand syndrome (AVWS) due to a monoclonal gammopathy of undetermined significance. Initially this case was diagnosed as congenital von Willebrand disease (VWD); however, re-examination of the medical history rendered a congenital bleeding disorder unlikely. A normal plasma von Willebrand factor (VWF) propeptide level and a very short half-life of VWF after a test infusion with factor VIII/VWF concentrate confirmed the diagnosis AVWS. Two major surgical procedures were successfully managed using high-dose intravenous immunoglobulin. The differential diagnosis with congenital VWD and the diagnostic and therapeutic approaches of AVWS are discussed. We conclude that the diagnosis of AVWS relies primarily on clinical suspicion and a careful bleeding history. A correct diagnosis is essential for optimal perioperative management and treatment of bleeding episodes.

journal_name

Am J Hematol

authors

Eikenboom JC,Tjernberg P,Van Marion V,Heering KJ

doi

10.1002/ajh.20760

subject

Has Abstract

pub_date

2007-01-01 00:00:00

pages

55-8

issue

1

eissn

0361-8609

issn

1096-8652

journal_volume

82

pub_type

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