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Hemosiderosis is deposition of hemosiderin in kidney tubular cells. It has been reported in different types of hemolytic anemia 1 .In hemochromatosis, hemosiderin accumulation in tubular epithelial cells has also been reported though its extremely rare. Iron overload maybe also triggered by iron administration and blood transfusions.2 Renal hemosiderosis is usually secondary to chronic intravascular hemolysis. It is usually an incidental finding in postmortem biopsies and it is not considered as leading to significant functional impairment 3. There are cases of hemochromatosis-associated IgA nephropathy in the literature 4. Both cases have been considered as secondary IgA nephropathy associated with liver disease. There is also a case report of RPGN associated with hemochromatosis 5. Diverse mechanisms of tubular injury have been proposed recently. Hemoglobin, an intratubular degradation product of erythrocytes, is often taken up by proximal tubular epithelial cells. Hemoglobin, heme, and iron are degraded and accumulate in the tubular cells, causing tubular injury through oxidative stress, inflammation, mitochondrial damage, and fibrosis 6
We describe a case of renal hemosiderosis secondary to intravascular hemolysis in a patient with aplastic anemia and paroxysmal nocturnal hemoglobinuria who is transfusion dependant almost monthly. The patient presented with proteinuria and rise in creatinine level, his kidney biopsy revealed iron deposition and a CKD with moderate chronicity score. We suggest that cases of chronic intravascular hemolysis and patients with recurrent blood transfusions accompanied with asymptomatic urinary abnormalities to have a renal biopsy to exclude underlying kidney pathology and predict potential renal insufficiency. Also initiating early treatment with iron chelation therapy may prevent kidney damage.
Case report: A40 years- old male patient referred to renal in August 2023 due to rise in creatinine and significant proteinuria. He is known with Aplastic anemia since 1999 failed ALG (Antitlymphocyte globulin ) treatment and received Tacrolimus and Cyclosporin with poor response. There was no donor option and he remained transfusion dependent (Monthly). He was also diagnosed with nocturnal paroxysmal hemoglobinuria {NPH] in 2005 and in 2016 he underwent splenectomy and cholecystectomy due to gall stones. He had persistent macrocytic aneamia, He was also diagnosed with Mesenteric vein thrombosis and was kept on life long anticoagulation. He had no history of Hypertension or Diabetes and he was not using any medication other than Folic acid and Rivaroxiban. He has had no iron chelation therapy. On examination he was pale, had a normal BP, his BMI was 17, Neurological examination and other systemic examination were normal.
Laboratory investigations were as follows:
Urine dipstix showed RBCs 3+, Protein 2+
ANA was negative, C3&C4 were normal, Hepatitis B and C serology and HIV were negative, Syphilis was negative as well. KUB USS showed normal size kidneys with increased echogenicity and reduced corticomedullary differentiation bilaterally suggestive of CKD.
Date
HB
WC
PLT
TBil
MCV
eGFR
CR
Ferritin
UPCR
ALT
GGT
Alb
16/8/23
6.1
6.8
193
49
116
73
110
77
87
12/7/23
7.1
7.8
272
48
92
90
0.125
112
131
40
14/6/23
7.5
7.4
172
34
83
98
9589
95
181
41
Percutaneous kidney biopsy was performed and there was a total of 18 glomeruli of which 2 globally sclerosed. There was no crescents or necrotizing lesions, Fibrosis Present (26-50%) and Lymphoplasmacytic inflammation: Present (26-50%). haemosiderin pigment in tubules (confirmed with Perl's Prussian blue stain) , Immunofluorescence studies was negative for IgA, IgG:,IgM, C3 and C1q. Total renal chronicity score: moderate (5/10).
The patient was diagnosed as renal hemosiderosis likely secondary to recurrent blood transfusion and hemolysis. He was referred back to hematology with no additional treatment.
Figure 1. H&E stain
Hemosiderosis associated renal involvement is extremely rare. In our case due to mild renal dysfunction and negative immuniflorecense we though the Renal haemosiderosis is likely secondary to recurrent blood transfusion and haemolysis. At this point there is no specific treatment other than giving iron chelating agents going forward., Bp control and standard care management for CKD to prevent progression.
Lesson: High iron load from hemolysis and recurrent blood transfusion has consequences including CKD hence iron chelation therapy has to be in place to avoid these sequences.
We suggest that cases of chronic intravascular hemolysis and patients with recurrent blood transfusions accompanied with asymptomatic urinary abnormalities to have a renal biopsy to exclude underlying kidney pathology and predict potential renal insufficiency.