2016
DOI: 10.1002/pbc.26246
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An 11‐year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre

Abstract: Wilms tumour (WT) is the commonest primary malignant renal tumour of childhood. Acquired von Willebrand syndrome (avWS) is a well-described paraneoplastic phenomenon, but it is uncommon and may not be detected until clinically significant bleeding is encountered during interventional procedures. Previous studies on small cohorts of patients have determined an incidence of between 4 and 8%. We have performed a retrospective study on cases of WT presenting over an 11.5-year period to a paediatric haematology/onc… Show more

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Cited by 13 publications
(3 citation statements)
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“…The laboratory findings are characterised by prolonged APTT, low factor VIII, and low von Willebrand factor (VWF) antigen and activity. The use of VWF concentrate is recommended for surgical procedures or bleeding, but the response may be variable until the underlying tumour is reduced (Fosbury et al , ).…”
Section: Management Of Thrombocytopenia and Coagulopathymentioning
confidence: 99%
“…The laboratory findings are characterised by prolonged APTT, low factor VIII, and low von Willebrand factor (VWF) antigen and activity. The use of VWF concentrate is recommended for surgical procedures or bleeding, but the response may be variable until the underlying tumour is reduced (Fosbury et al , ).…”
Section: Management Of Thrombocytopenia and Coagulopathymentioning
confidence: 99%
“…Methylation inactivation of enriched tumor suppressor genes such as ADCY4 [145], FBLN1 [146], FBN2 [147], ADAMTS9 [148], NELL2 [149] and PCDH18 [150] were important for progression of various cancers such as breast cancer, colorectal cancer, nasopharyngeal cancer and kidney cancer, but loss of these genes may be linked with development of WT. Enriched genes such as VWF (Von Willebrand factor) [151] and ACVR2B [152] were identi ed with progression of WT. ITPR1 was linked with activation of autophagy in kidney cancer [153], but this gene may be responsible for induction of autophagy in WT.…”
Section: Discussionmentioning
confidence: 99%
“…One of the more common pediatric malignancies associated with AvWS is Wilms' tumor [12]. A retrospective evaluation of the coagulation profile of 190 children with Wilms' tumor revealed AvWS in eight for an incidence of approximately 4% [13].…”
Section: Discussionmentioning
confidence: 99%