title: Acquired von Willebrand syndrome and Wilms tumor: not always benign.
abstract: Current literature suggests that acquired von Willebrand syndrome associated with Wilms tumor (AVWS-WT) occurs infrequently and usually has little clinical significance. Treatment strategies are thus poorly defined. We describe two patients with AVWS-WT and profuse bleeding who required intensive multimodal therapy, including aggressive blood component and factor replacement and plasmapheresis. They achieved adequate surgical hemostasis only after the renal vessels were ligated, with resolution of the coagulopathy upon tumor removal. Our experience suggests that AVWS-WT is not always benign. A careful bleeding history should always be obtained in patients with suspected renal tumors for consideration of pre-operative screening for AVWS.
authors.affiliation: Department of Pediatrics, Hematology-Oncology Section, Baylor College of Medicine, Houston, Texas 77030, USA.
authors.forename: Patricia A
authors.initials: PA
authors.lastname: Baxter
authors.forename: Jed G
authors.initials: JG
authors.lastname: Nuchtern
authors.forename: Robert P
authors.initials: RP
authors.lastname: Guillerman
authors.forename: Donald H
authors.initials: DH
authors.lastname: Mahoney
authors.forename: Jun
authors.initials: J
authors.lastname: Teruya
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
authors.forename: Donald L
authors.initials: DL
authors.lastname: Yee
mesh_terms: D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007700:Kinetics; D014057:Tomography, X-Ray Computed; D009396:Wilms Tumor / Q000150:complications* / Q000188:drug therapy / Q000473:pathology* / Q000601:surgery; D014842:von Willebrand Diseases / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology* / Q000628:therapy