title: How I treat the acquired von Willebrand syndrome.
abstract: The acquired von Willebrand syndrome (AVWS) is a bleeding disorder that is frequently unrecognized or is misdiagnosed as von Willebrand disease. AVWS is characterized by structural or functional defects of von Willebrand factor (VWF) that are secondary to autoimmune, lymphoproliferative or myeloproliferative, malignant, cardiovascular, or other disorders. VWF abnormalities in these disorders can result from (1) antibody-mediated clearance or functional interference, (2) adsorption to surfaces of transformed cells or platelets, or (3) increased shear stress and subsequent proteolysis. Diagnosis can be challenging as no single test is usually sufficient to prove or exclude AVWS. Furthermore, there are no evidence-based guidelines for management. Treatments of the underlying medical condition, including chemo/radiotherapy, surgery, or immunosuppressants can result in remission of AVWS, but is not always feasible and successful. Because of the heterogeneous mechanisms of AVWS, more than one therapeutic approach is often required to treat acute bleeds and for prophylaxis during invasive procedures; the treatment options include, but are not limited to, desmopressin, VWF-containing concentrates, intravenous immunoglobulin, plasmapheresis or recombinant factor VIIa. Here, we review the management of AVWS with an overview on the currently available evidence and additional considerations for typical treatment situations.
authors.affiliation: Department of Hematology, Hemostasis, Oncology and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany. tiede.andreas@mh-hannover.de
authors.forename: Andreas
authors.initials: A
authors.lastname: Tiede
authors.forename: Jacob H
authors.initials: JH
authors.lastname: Rand
authors.forename: Ulrich
authors.initials: U
authors.lastname: Budde
authors.forename: Arnold
authors.initials: A
authors.lastname: Ganser
authors.forename: Augusto B
authors.initials: AB
authors.lastname: Federici
mesh_terms: D000818:Animals; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D015942:Factor VIIa / Q000627:therapeutic use; D006402:Hematologic Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D006490:Hemostatics / Q000627:therapeutic use*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D010956:Plasmapheresis; D011994:Recombinant Proteins / Q000627:therapeutic use; D013577:Syndrome; D014842:von Willebrand Diseases / Q000175:diagnosis* / Q000453:epidemiology / Q000473:pathology / Q000628:therapy*; D014841:von Willebrand Factor / Q000627:therapeutic use
chemical_list: D006490:Hemostatics; D016756:Immunoglobulins, Intravenous; D011994:Recombinant Proteins; D014841:von Willebrand Factor; C103587:recombinant FVIIa; D015942:Factor VIIa; D003894:Deamino Arginine Vasopressin