Focal segmental glomerulosclerosis in a patient with polycythemia vera

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  • IYODA Masayuki
    Department of Nephrology, Showa University School of Medicine
  • ITO Jyun
    Department of Nephrology, Showa University School of Medicine
  • AJIRO Yuriko
    Department of Nephrology, Showa University School of Medicine
  • NAGAI Hisako
    Department of Nephrology, Showa University School of Medicine
  • UCHIDA Junichi
    Department of Nephrology, Showa University School of Medicine
  • HONDA Hirokazu
    Department of Nephrology, Showa University School of Medicine
  • KUROKI Aki
    Department of Nephrology, Showa University School of Medicine
  • SHIBATA Takanori
    Department of Nephrology, Showa University School of Medicine
  • KITAZAWA Kozo
    Department of Nephrology, Showa University School of Medicine
  • SUGISAKI Tetsuzo
    Department of Nephrology, Showa University School of Medicine

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Other Title
  • 真性多血症に巣状糸球体硬化症を発症し血液透析導入に至った１例
  • ショウレイ シンセイ タケツショウ ニ ソウジョウ シキュウタイ コウカショウ オ ハッショウ シ ケツエキ トウセキ ドウニュウ ニ イタッタ 1レイ

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Abstract

Herein we describe the case of a patient with focal segmental glomerulosclerosis (FSGS) following polycythemia vera (PV) on whom hemodialysis was started 7 years later. A 66-year-old woman who had been treated for PV with hydroxyurea and phlebotomy for three years was referred to our hospital because of nephrotic syndrome. Renal biopsy performed at her local hospital revealed FSGS. Although she had received prednisolone at an initial dose of 45mg/day, no significant improvement of proteinuria was achieved. The dose of prednisolone was tapered because the second renal biopsy revealed sclerosing glomerulopathy. We considered that FSGS was associated with PV because renal hemodynamic alterations in PV could result in FSGS as in any other secondary FSGS and there was no proteinuria at the initial detection of PV. On January 29, 1999, she developed massive proteinuria (9.6g/day) and the findings of the third renal biopsy worsened in comparison with that of the first renal biopsy. Thereafter, hydroxyurea or ranimustine was used in treating PV at an outpatient clinic. However severe thrombocytosis was difficult to control, and progressive renal dysfunction finally necessitated hemodialysis on January 18, 2005. In conclusion, physicians should be aware of the risk of progressive renal failure in patients with FSGS following PV, particularly in patients with persistent thrombocytosis.

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