Related Experiment Videos
Acquired von Willebrand disease in Wilms' tumor patients
M J Coppes1, S W Zandvoort, C R Sparling
1Department of Paediatrics, Hospital for Sick Children, Toronto, Canada.
Insights
Eight percent of children with new Wilms' tumor diagnoses had acquired von Willebrand disease (vWD). This finding highlights the need for further research into vWD in pediatric cancer patients.
Area of Science:
- Pediatric Oncology
- Hematology
- Clinical Research
Background:
- Wilms' tumor is a common pediatric kidney cancer.
- Acquired von Willebrand disease (vWD) is a rare bleeding disorder.
- The association between Wilms' tumor and acquired vWD is not well-established.
Observation:
- A prospective study evaluated 50 children with newly diagnosed Wilms' tumor.
- Comprehensive bleeding histories and laboratory tests were performed.
- vWF multimer analysis was conducted when vWD was suspected.
Findings:
- Acquired vWD was identified in 8% (4 out of 50) of the children.
- Two patients presented with type III vWD, and two with type I vWD.
- These findings suggest a notable incidence of acquired vWD in this pediatric population.
Implications:
- Further large-scale prospective trials are warranted to confirm these findings.
- Standardized diagnostic protocols including bleeding history and specific laboratory tests are recommended.
- Testing the efficacy of 1-desamino-8-D-arginine vasopressin (DDAVP) in managing acquired vWD in Wilms' tumor patients is crucial to potentially avoid blood product transfusions.
Purpose:
A prospective study was performed to determine the incidence of acquired von Willebrand disease (vWD) in children with newly diagnosed Wilms' tumor.
Patients And Methods:
Fifty consecutive children with newly diagnosed Wilms' tumor were evaluated. Detailed family and bleeding histories were obtained in all cases. Laboratory evaluation included measurement of the circulating platelet count, bleeding time (BT), factor VIII (FVIII) and von Willebrand factor (vWF) levels, and ristocetin cofactor (RCoF) activity. A vWF multimer analysis was obtained in all cases in which vWD was suspected.
Results:
Four of 50 (8%) consecutive children with a diagnosis of Wilms' tumor were found to have acquired vWD. Laboratory findings indicated type III vWD in two patients and type I vWD in the other two.
Conclusions:
The incidence of acquired vWD in association with Wilms' tumor merits further study through a large prospective trial. Such a trial should include careful family and clinical bleeding histories plus measurement of a platelet count, BT, coagulant FVIII and vWF levels, RCoF activity, and vWF multimer analysis. The response to 1-desamino-8-D-arginine vasopressin (DDAVP) should be tested in all patients with Wilms' tumor and acquired vWD, including patients with a type III profile, before an invasive procedure is performed. Successful use of DDAVP may avoid exposure of affected patients to blood products.