An 11-year experience of acquired von Willebrand syndrome in children diagnosed with Wilms tumour in a tertiary referral centre.
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.
Katharine Dormandy Haemophilia and Thrombosis Centre, Royal Free London NHS Foundation Trust, London, UK.
Emma
E
Fosbury
The Institute of Cancer Research, London, UK.|Department of Paediatric Oncology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Elwira
E
Szychot
Department of Paediatric Oncology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Olga
O
Slater
Haemophilia Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Mary
M
Mathias
Haemophilia Centre, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Keith
K
Sibson
D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006470:Hemorrhage / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007680:Kidney Neoplasms / Q000503:physiopathology*; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D006113:United Kingdom / Q000453:epidemiology; D009396:Wilms Tumor / Q000503:physiopathology*; D014842:von Willebrand Diseases / Q000175:diagnosis / Q000453:epidemiology*
Wilms tumour; acquired von Willebrand syndrome; children; factor VIII/VWF concentrate