Acquired von willebrand syndrome and Wilms Tumor: Not always benign

Patricia A. Baxter, Jed G. Nuchtern, Robert P. Guillerman, Donald H. Mahoney, Jun Teruya, Murali Chintagumpala, Donald L. Yee

Research output: Contribution to journalArticlepeer-review

23 Scopus citations


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 hemo-stasis 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.

Original languageEnglish (US)
Pages (from-to)392-394
Number of pages3
JournalPediatric Blood and Cancer
Issue number3
StatePublished - Mar 2009


  • Acquired von willebrand syndrome
  • Von willebrand factor
  • Wilms tumor

ASJC Scopus subject areas

  • Oncology
  • Pediatrics, Perinatology, and Child Health
  • Hematology
  • Medicine(all)


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