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1 March 2012 - Case of the Week #235
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Thanks to Dr. Saroona Haroon, The Aga Khan University Hospital (Pakistan), for contributing this case. To contribute a Case of the Week, follow the guidelines on our Case of the Week page.
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Case of the Week #235
A 52 year old Asian man, hypertensive for 7 years, presented with slowly increasing serum creatinine levels, currently 3.1 mg/dl, and current renal failure. Urine showed microscopic hematuria. His past medical history included a heart valve replacement due to valvular heart disease at age 30 years. As the etiology of the increasing creatinine was unknown, a renal biopsy was obtained.
What is your diagnosis?
Renal hemosiderosis, secondary to hemolytic anemia (heart valve complication)
The diagnosis was confirmed by an iron stain:
Renal hemosiderosis due to hemolytic anemia is a rare cause of renal failure (Am J Pathol 1966;48:409, Indian J Pathol Microbiol 2011;54:379). Hemosiderosis is defined as the histologic equivalent of secondary iron overload, usually due to hemosiderin, which may be focal. Hemosiderin is a golden yellow to brown granular cellular pigment due to hemoglobin breakdown. Typically, free serum hemoglobin is bound to haptoglobin, but in severe cases, the free hemoglobin is filtered by the kidney and deposited in the proximal convoluted tubules, which may cause renal failure.
Renal hemosiderosis is caused by recurrent intravascular hemolysis, and may be due to hemolytic anemia, paroxysmal nocturnal hemoglobinuria (PNH) and mechanical hemolysis from prosthetic cardiac valves. In this case, the hemolysis was attribute to chronic mechanical damage due to shearing of red blood cells by the prosthetic valve. Correction of a dysfunctional valve may be therapeutic (Arch Mal Coeur Vaiss 1993;86:111).
Nat Pernick, M.D., President
and Liz Parker, B.A., Associate Medical Editor
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