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Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature

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Abstract

Renal involvement in paroxysmal nocturnal hemoglobinuria (PNH) is usually clinically not apparent but, in cases with clinical involvement, varies from reversible acute dysfunction to chronic irreversible damage. We report two cases of acute kidney injury and one case of chronic kidney disease due to PNH. In all three cases a diagnosis of PNH was made after detection of renal dysfunction. The renal involvement was documented on both histology and on imaging studies. Finally, we briefly review the main literature data on renal involvement in PNH.

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References

  1. Rubin H (1971) Paroxysmal nocturnal hemoglobinuria with renal failure. JAMA 215(3):433–436

    Article  PubMed  CAS  Google Scholar 

  2. Chow KM, Lai FM, Wang AY, Chan YL, Tang NL, Li PK (2001) Reversible renal failure in paroxysmal nocturnal hemoglobinuria. Am J Kidney Dis 37(2):E17

    Article  PubMed  CAS  Google Scholar 

  3. Zachee P, Henckens M, Van Damme B, Boogaerts MA, Rigauts H, Verberckmoes RK (1993) Chronic renal failure due to renal hemosiderosis in a patient with paroxysmal nocturnal hemoglobinuria. Clin Nephrol 39(1):28–31

    PubMed  CAS  Google Scholar 

  4. Clark DA, Butler SA, Braren V, Hartmann RC, Jenkins DE Jr (1981) The kidneys in paroxysmal nocturnal hemoglobinuria. Blood 57(1):83–89

    PubMed  CAS  Google Scholar 

  5. Crosby WH (1953) Paroxysmal nocturnal hemoglobinuria: relation of the clinical manifestations to underlying pathogenic mechanisms. Blood 8(9):769–812

    PubMed  CAS  Google Scholar 

  6. Zeidman A, Chagnac A, Wisnovitz M, Mittelman M (1994) Hemolysis-induced acute renal failure in paroxysmal nocturnal hemoglobinuria. Nephron 66(1):112

    PubMed  CAS  Google Scholar 

  7. Mooraki A, Boroumand B, Mohammad Zadeh F, Ahmed SH, Bastani B (1998) Acute reversible renal failure in a patient with paroxysmal nocturnal hemoglobinuria. Clin Nephrol 50(4):255–257

    PubMed  CAS  Google Scholar 

  8. Jackson GH, Noble RS, Maung ZT, Main J, Smith SR, Reid MM (1992) Severe haemolysis and renal failure in a patient with paroxysmal nocturnal haemoglobinuria. J Clin Pathol 45(2):176–177

    Article  PubMed  CAS  Google Scholar 

  9. Sechi LA, Marigliano A, Tedde R (1988) Venous thrombosis and acute renal failure in paroxysmal nocturnal haemoglobinuria. Nephrol Dial Transplant 3(5):708–709

    PubMed  CAS  Google Scholar 

  10. Botelho RJ, Ebrahim SB, Sainsbury R, Bendall MJ (1981) Paroxysmal nocturnal haemoglobinuria with renal, infective and immunological abnormalities. Postgrad Med J 57(673):736–738

    Article  PubMed  CAS  Google Scholar 

  11. Jose MD, Lynn KL (2001) Acute renal failure in a patient with paroxysmal nocturnal hemoglobinuria. Clin Nephrol 56(2):172–174

    PubMed  CAS  Google Scholar 

  12. Vanwallghem J, Zachee P, Kuypers D, Maes B, Coosemans W, Pirenne J, Vanrenterghem Y (1998) Renal transplantation for end stage renal disease due to paroxysmal nocturnal hemoglobinuria. Nephrol Dial Transplant 13(12):3250–3252

    Article  Google Scholar 

  13. Rimola J, Martin J, Puig J, Darnell A, Massuet A (2004) The kidney in paroxysmal nocturnal haemoglobinuria: MRI findings. Br J Radiol 77(923):953–956

    Article  PubMed  CAS  Google Scholar 

  14. Versvijwel G, Vanbeckevoort D, Maes B, Oyen R (1999) Paroxysmal nocturnal hemoglobinuria: MRI of renal cortical hemosiderosis in two patients, including one renal transplant. Nephrol Dial Transplant 14(6):1586–1589

    Article  Google Scholar 

  15. Braun SR, Weiss FR, Keller AI, Ciccone JR, Preuss HG (1970) Evaluation of the renal toxicity of heme proteins and their derivatives: a role in the genesis of acute tubular necrosis. J Exp Med 131(3):443–460

    Article  PubMed  CAS  Google Scholar 

  16. Zager RA, Gamelin LM (1989) Pathogenetic mechanisms in experimental hemoglobinuric acute renal failure. Am J Physiol 256(3 Pt 2):F446–F455

    PubMed  CAS  Google Scholar 

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Correspondence to Alok Sharma.

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Nair, R.K., Khaira, A., Sharma, A. et al. Spectrum of renal involvement in paroxysmal nocturnal hemoglobinuria: report of three cases and a brief review of the literature. Int Urol Nephrol 40, 471–475 (2008). https://doi.org/10.1007/s11255-008-9356-5

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  • DOI: https://doi.org/10.1007/s11255-008-9356-5

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