Acquired von Willebrand syndrome: Diagnostic problems and therapeutic options

• Published in: American journal of hematology Vol. 82; no. 1; pp. 55 - 58
• Main Authors: Eikenboom, Jeroen C.J., Tjernberg, Pernilla, Van Marion, Vincent, Heering, Karel J.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.01.2007; Wiley-Liss
• Subjects: Aged; Biological and medical sciences; Diagnosis, Differential; Hematologic and hematopoietic diseases; Hemorrhage - blood; Hemorrhage - diagnosis; Hemorrhage - drug therapy; Hemorrhage - etiology; Humans; immunoglobulin; Immunoglobulins, Intravenous - administration & dosage; Immunologic Factors - administration & dosage; Male; Medical sciences; Platelet diseases and coagulopathies; propeptide; Syndrome; von Willebrand disease; von Willebrand Diseases - blood; von Willebrand Diseases - diagnosis; von Willebrand Diseases - drug therapy; von Willebrand Diseases - etiology; von Willebrand factor; von Willebrand Factor - analysis; Immunoglobulins; Hematology; immunoglobulin; Acquired; Willebrand disease; Propeptide; Hemopathy; Genetic disease; Treatment; von Willebrand factor; von Willebrand disease; Diagnosis; Willebrand factor; Coagulopathy
• ISSN: 0361-8609; 1096-8652
• DOI: 10.1002/ajh.20760

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.