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Platelet dysfunction associated with Wilms tumor and hyaluronic acid
American Journal of Hematology
|March 1, 1987
Summary
Nephroblastoma can cause acquired von Willebrand disease due to high hyaluronic acid levels, leading to hyperviscosity and coagulation issues. Treatment of the tumor resolved these hematologic abnormalities.
Area of Science:
- Oncology
- Hematology
- Biochemistry
Background:
- Acquired von Willebrand disease (AVWD) is a rare bleeding disorder.
- Nephroblastoma is a pediatric kidney cancer.
- Previous reports suggest a link between nephroblastoma and AVWD.
Observation:
- A patient with nephroblastoma presented with coagulopathy mimicking AVWD.
- The patient had low levels of von Willebrand factor (vWF) and Factor VIII activity.
- Elevated serum hyaluronic acid (HA) levels and hyperviscous serum were observed, with HA found in the tumor matrix.
Findings:
- Hyaluronic acid (HA) interfered with assays for von Willebrand factor antigen (F.VIIIR:Ag).
- In vitro, HA reduced F.VIII:C activity and impaired platelet aggregation.
- Chemotherapy and tumor removal normalized HA levels and corrected coagulation abnormalities.
- Animal studies with HA mimicked the patient's coagulation defects.
Implications:
- Elevated HA in nephroblastoma patients can cause hyperviscosity and spurious AVWD.
- This finding clarifies the mechanism of coagulopathy in some nephroblastoma cases.
- Understanding this link is crucial for accurate diagnosis and management of bleeding disorders in cancer patients.
