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Polycythemia Rubra Vera and Sporadic Bilateral Renal Angiomyolipomas: A Case Report

James C Barton1,2,3, Barrett P Cary4, Robert M Frederickson3

  • 1Department of Medicine, University of Alabama at Birmingham, Birmingham, USA.

Cureus
|May 16, 2022
PubMed

Insights

This study reports a rare case of simultaneous Polycythemia rubra vera (PRV) and renal angiomyolipomas (AMLs) in a 71-year-old woman. Management involved PRV treatment and AML surveillance, highlighting rare co-occurrences.

Area of Science:

  • Hematology
  • Oncology
  • Nephrology

Background:

  • Polycythemia rubra vera (PRV) is a myeloproliferative neoplasm causing excess blood cell production.
  • Renal angiomyolipomas (AMLs) are typically sporadic benign tumors.

Observation:

  • A 71-year-old woman presented with synchronous diagnoses of PRV and asymptomatic bilateral renal AMLs.
  • The patient received phlebotomy and hydroxyurea for PRV, with surveillance for AMLs.

Findings:

  • This case represents a rare co-occurrence of PRV and sporadic renal AMLs.
  • Comparison with two similar reported cases provides insights into management and potential genetic links.

Implications:

  • Understanding the synchronous occurrence of PRV and AMLs can inform clinical management strategies.
  • Further research into the acquired genetic basis may reveal shared pathways or risk factors.
Keywords:
angiomyolipomaerythrocytosiserythropoietinpolycythemia rubra verasomatic mutation

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