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



(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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