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An 11‐year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre
Author(s) -
Fosbury Emma,
Szychot Elwira,
Slater Olga,
Mathias Mary,
Sibson Keith
Publication year - 2017
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.26246
Subject(s) - medicine , tertiary referral centre , incidence (geometry) , wilms tumour , referral , pediatrics , retrospective cohort study , tertiary referral hospital , tertiary care , hematology , wilms' tumor , family medicine , physics , optics
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/oncology unit in a tertiary referral centre to review the incidence of avWS, bleeding phenotype, management, and response to treatment of the primary pathology.