Renal Hemosiderosis Due to Thalassemia: A Light and Electron Microscopy Study with Electron Probe X-Ray Microanalysis

Abstract

A kidney biopsy specimen with pronounced hemosiderosis from a patient with beta-thalassemia major was studied by light and electron microscopy, including X-ray microanalysis. Ferritin was absorbed from the glomerular ultrafiltrate through the parietal epithelial cells and the tubular epithelial cells and from the blood through the endothelial cells. It was transported in siderosomes into the surrounding basal lamina, where electron-dense deposits of hemosiderin were found in the outer part of the lamina densa and the reticular lamina. Fibrosis was seen as a reaction to the iron followed by severe atrophy of affected structures.

Magnetic resonance detection of kidney iron deposition in sickle cell disease: A marker of chronic hemolysis

“…Reticulocyte counts and LDH levels were normal in our thalassemia patients, in marked contrast to those with SCD. But despite our failure to find radiographically-detectable iron, a number of investigators have claimed that iron damages thalassemic kidneys(16–19,37,38). Particularly, proximal tubular dysfunction, common in children and adults with thalassemia, correlates with transfusional iron burden(17,37).…”

Iron overload and urinary lysosomal enzyme levels in β-thalassaemia major

“…Both structural and functional changes that are generally thought to occur late in life have also been observed in kidneys of thalassaemic patients [7,14,15] and iron accumulation in siderosomes, i.e. secondary iron containing lysosomes, has been shown [6]. The problem however has not been studied as extensively as in the case of the liver.…”

Blue kidney in a pale patient--a case for a causal association between renal haemosiderosis in paroxysmal nocturnal haemoglobinuria and chronic kidney disease

“…Renal impairment associated with mesangial expansion, hypercellularity, glomerulosclerosis, interstitial fibrosis and tubular atrophy has been reported in animal models of experimental haemosiderosis [ 8 ] as well as in patients with beta thalassaemia [ 9 ]. The prevailing hypothesis for chronic iron overload-induced nephrotoxicity is that of an oxidant injury to cellular membrane phospholipids by reactive oxygen species (ROS).…”