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CASE REPORT  
Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 379-380
Refractory anemia leading to renal hemosiderosis and renal failure


1 Department of Pathology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore, India
2 Department of Microbiology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore, India
3 Department of Biochemistry, Institute of Nephrourology, Victoria Hospital Campus, Bangalore, India
4 Department of Nephrology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore, India

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Date of Web Publication27-May-2011
 

   Abstract 

Renal hemosiderosis is a rare cause of renal failure and, as a result, may not be diagnosed unless a detailed history, careful interpretation of blood parameters and renal biopsy with special staining is done. Here, we present a rare case of renal hemosiderosis presenting with renal failure.

Keywords: Hemosiderosis, renal biopsy, renal failure

How to cite this article:
Siddappa S, Mythri K M, Kowsalya R, Parekh A. Refractory anemia leading to renal hemosiderosis and renal failure. Indian J Pathol Microbiol 2011;54:379-80

How to cite this URL:
Siddappa S, Mythri K M, Kowsalya R, Parekh A. Refractory anemia leading to renal hemosiderosis and renal failure. Indian J Pathol Microbiol [serial online] 2011 [cited 2014 Dec 18];54:379-80. Available from: http://www.ijpmonline.org/text.asp?2011/54/2/379/81648



   Introduction Top


Hemosiderosis is defined as the histologic equivalent of secondary iron overload, usually in the form of hemosiderin, which may be local, i.e. restricted to one organ, or generalized. It is often associated with diseases involving chronic, extensive destruction of red blood cells, such as thalassemia major. Multiple blood transfusions in cases of refractory anemia have been associated with the development of hemosiderosis of the liver, spleen and other organs. [1] Regularly transfused patients can easily become iron overloaded. [2] Normally, deposition of hemosiderin, the storage form of iron in the parenchymal cells, is not known to cause organ damage. [3] Only few cases have been reported in the literature involving the deposition of hemosiderin in kidneys with renal failure. [4],[5]


   Case History Top


A 50-year-old male presented with features of worsening of renal function over the past 15 days. He had a history of repeated blood transfusion (about two transfusions per month) over the past 2 years for chronic refractory anemia. There was no history of cardiac valve replacement. Urinalysis showed acidic pH with proteinuria -1 + (30 mg/dl), hematuria and presence of leucocytes. The biochemical parameters were as follows: blood urea nitrogen - 80 mg/dl, serum creatinine - 5.0 mg/dl, total iron binding capacity - 216 μg/dl (228-428 μg/dl), transferrin saturation 44.4% (13-45%) and serum ferritin - 826.7 ng/ml (30-400 ng/ml). Liver function test showed a two-fold rise in the alanine aminotransferase/asparate aminotransferase levels with decrease in total protein levels (3 g/dl).

His hemoglobin was 6.9 g/dl with a peripheral smear showing dimorphic anemia. The bone marrow showed a hypercellular marrow. Protein electrophoresis showed no abnormal spikes. Ultrasonography showed presence of fatty liver.

The renal biopsy of left kidney showed patent glomeruli with mild mesangial expansion and obliteration of bowmen's space. The interstitium showed variable degree of fibrosis and ectatically dilated tubules filled with proteineous casts. The most striking feature was deposition of golden-brown pigment within the cytoplasm of the tubular epithelium, which was intense and distributed throughout in all sections examined. This pigment was seen plugging the tubules and was also present within the tubular cells. Another significant finding was that the glomeruli were all spared from pigment deposition [Figure 1].
Figure 1: Renal biopsy: Prussian blue stain - Arrow showing glomeruli spared of the deposition (Prussian blue, ×200)

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Immunofluorescence staining for Ig A, M, G, C3 and C1q were all negative. Prussian blue staining performed was positive. [Figure 2] shows the reticulocyte stain positivity of 8%. Urine sample stained for presence of hemosiderin was negative. Additional tests like sucrose hemolysis test and Ham's test for acid hemolysis could not be performed as the patient was discharged against medical advice and was lost to follow-up.
Figure 2: Retic stain 8% (Supravital Stain, ×1000)

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   Discussion Top


The pathological diagnosis was renal hemosiderosis with renal failure. Pathologic findings in a patient with renal failure and renal hemosiderosis have been reported as tubular damage, slight chronic tubulointerstitial inflammation and fibrosis. Hemosiderin is hemoglobin derived, golden yellow to brown granular or crystalline pigment in which form iron is stored in the cells. [6] Intravascular hemolysis increases free hemoglobin in circulation, which binds to haptoglobin. But, in severe hemolysis, the free hemoglobin is filtered by the kidney as opposed to hemoglobin bound to haptoglobin and reabsorbed by and deposited in the proximal convoluted tubules. The exact pathogenesis is not known. The chemically active iron in hemosiderin may cause free radical-mediated injury through Fenton reaction, causing tubular damage and, eventually, cell death. [7] The deposits in a setting of hypovolemia and acidic urine can lead to renal failure.

In the above case, with history of repeated transfusion and no appropriate iron reduction therapy, the chances of iron overload are very high. Thus, a setting of poor hemodynamic state and iron overload may have precipitated renal failure.

Additional tests like sucrose hemolysis test and Ham's test for acid hemolysis were required to rule out other causes of renal hemosiderosis like paroxysmal nocturnal hemoglobinuria, which could not be performed as the patient was discharged against medical advice and was lost to follow-up.

 
   References Top

1.Wyatt JP, Goldenberg H. Hemosiderosis in refractory anemia. Arch Intern Med 1949;83:67-76.   Back to cited text no. 1
    
2.Porter JB. Practical management of iron overload. Br J Haematol 2001;115:239-52.  Back to cited text no. 2
    
3.Siegelman ES, Mitchell DG, Semelka RC. Abdominal iron deposition: metabolism, MR findings, and clinical importance. Radiology 1996;99:13-22.  Back to cited text no. 3
    
4.Ackerman D, Vogt B, Gugger M, Marti HP. Renal hemochromatosis: An unusual presentation of acute renal failure in a patient following heart valve prosthesis. Nephrol Dial Transplant 2004;19:2682-3.  Back to cited text no. 4
    
5.Kümpers P, Herrmann A, Lotz J, Mengel M, Schwarz A. A blue kidney-chronic renal failure as a consequence of siderosis in paroxysmal nocturnal hemoglobinuria? Clin Nephrol 2006;66:210-3.  Back to cited text no. 5
    
6.Kumar V, Abbas AK, Fausto N, Aster JC. Cellular Adaptations, Cell Injury, and Cell Death. In: Kumar V, Abbas AK, Fausto N, Aster JC, editors. Robbins and Cotran Pathologic basis of disease. 8 th ed. Philadelphia: Saunders; 2010. p. 36.  Back to cited text no. 6
    
7.Zhou X, Laszik Z, Wang X, Silva F, Vaziri N. Association of renal injury with increased oxygen free radical activity and altered nitric oxide metabolism in chronic experimental hemosiderosis. Lab Invest 2000;80:1905-4.  Back to cited text no. 7
    

Top
Correspondence Address:
Sujatha Siddappa
Department of Pathology, Institute of Nephrourology, Victoria Hospital Campus, Bangalore
India
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DOI: 10.4103/0377-4929.81648

PMID: 21623097

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