A patient with a Wilms tumor may develop an acquired von Willebrand's syndrome (AVWS).
Clinical features:
(1) absence of a family history of von Willebrand's disease
(2) absence of a personal history of von Willebrand's disease in the remote past
(3) onset of a bleeding disorder with features of von Willebrand's disease
(4) diagnosis of a Wilms tumor
(5) remission of the AVWS after therapy of the tumor
The patient may present with:
(1) abnormal bruising
(2) abnormal bleeding
(3) a prolonged aPTT
Observations:
(1) This occurrence of AVWS may be more common in children with bilateral Wilms tumors.
(2) A child with Wilms tumor should be screened for abnormal coagulation, especially prior to surgery.
(3) A child with the new onset of von Willebrand's disease should be screened for Wilms tumor.
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Specialty: Hematology Oncology, Clinical Laboratory
ICD-10: ,