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Acquired von Willebrand syndrome: Diagnostic problems and therapeutic options
Author(s) -
Eikenboom Jeroen C.J.,
Tjernberg Pernilla,
Van Marion Vincent,
Heering Karel J.
Publication year - 2007
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.20760
Subject(s) - von willebrand disease , medicine , von willebrand factor , medical history , differential diagnosis , pediatrics , monoclonal gammopathy of undetermined significance , intensive care medicine , pathology , monoclonal , immunology , antibody , monoclonal antibody , platelet
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. Am. J. Hematol., 2006. © 2006 Wiley‐Liss, Inc.