title: 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.
authors.affiliation: Department of Hematology, Hemostasis and Thrombosis Research Center, Leiden University Medical Center, Leiden, The Netherlands. h.c.j.eikenboom@LUMC.nl
authors.forename: Jeroen C J
authors.initials: JC
authors.lastname: Eikenboom
authors.forename: Pernilla
authors.initials: P
authors.lastname: Tjernberg
authors.forename: Vincent
authors.initials: V
authors.lastname: Van Marion
authors.forename: Karel J
authors.initials: KJ
authors.lastname: Heering
mesh_terms: D000368:Aged; D003937:Diagnosis, Differential; D006470:Hemorrhage / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage*; D007155:Immunologic Factors / Q000008:administration & dosage*; D008297:Male; D013577:Syndrome; D014842:von Willebrand Diseases / Q000097:blood / Q000175:diagnosis* / Q000188:drug therapy* / Q000209:etiology; D014841:von Willebrand Factor / Q000032:analysis
chemical_list: D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D014841:von Willebrand Factor