Polycythemia vera concomitant with renal angiomyolipoma: case report and clinical outcome
Article Category: Clinical vignette
Published Online: Aug 31, 2017
Page range: 89 - 92
DOI: https://doi.org/10.5372/1905-7415.1101.543
Keywords
© 2017 Supat Chamnanchanunt, Pravinwan Thungthong, Niphon Praditphol, Kunapa Iam-arunthai, Jirapong Dowreang, Chajchawan Nakhakes, Tawatchai Suwanban
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 3.0 License.
Background
Polycythemia vera has been found in a wide range of hematopoietic tissue disorders, but rarely concomitant with renal abnormality. To our knowledge there is only one available report of a patient with polycythemia vera concomitant with renal angiomyolipoma, and the report does not cover the clinical course of this rare finding.
Objectives
To report the presentation and clinical course of a patient with polycythemia vera concomitant with right renal angiomyolipoma.
Methods
We retrospectively reviewed the medical records of a patient with polycythemia vera who presented with renal angiomyolipoma.
Results
Our patient presented with a right renal mass and polycythemia vera diagnosed by a point mutation in the gene for Janus kinase 2. The mass was diagnosed as renal angiomyolipoma on histopathology. After partial nephrectomy, she was prescribed a low dosage of hydroxyurea without the return of erythrocytosis for a year.
Conclusions
Polycythemia vera responded well to low dosage hydroxyurea, which controlled erythrocytosis after removing the renal mass.