Acquired von Willebrand syndrome: an update.
Acquired von Willebrand syndrome (aVWS) is a rare bleeding disorder with laboratory findings similar to those for congenital von Willebrand disease (VWD). However, unlike congenital VWD, it arises in individuals with no personal or family history of bleeding. aVWS occurs in association with a variety of underlying disorders, most frequently in lymphoproliferative disorders, myeloproliferative disorders, and cardiovascular diseases. Through an analysis of the more recent literature data, the pathophysiology and the clinical, laboratory, and therapeutic aspects of this syndrome are concisely reported in this review.
Franchini
Massimo
M
Servizio di Immunoematologia e Trasfusione-Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy. mfranchini@univr.it
Lippi
Giuseppe
G
D051722:ADAM Proteins / Q000378:metabolism; D000071120:ADAMTS13 Protein; D000327:Adsorption; D001323:Autoantibodies / Q000276:immunology; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology; D001780:Blood Coagulation Tests; D002318:Cardiovascular Diseases / Q000150:complications; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D004700:Endocrine System Diseases / Q000150:complications; D005169:Factor VIII / Q000627:therapeutic use; D006402:Hematologic Diseases / Q000150:complications; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D014842:von Willebrand Diseases / Q000097:blood / Q000139:chemically induced / Q000175:diagnosis / Q000209:etiology* / Q000276:immunology / Q000503:physiopathology / Q000628:therapy; D014841:von Willebrand Factor / Q000737:chemistry / Q000276:immunology / Q000378:metabolism / Q000627:therapeutic use
D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D014841:von Willebrand Factor; C078147:F8 protein, human; D005169:Factor VIII; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human; D003894:Deamino Arginine Vasopressin