Internal Distribution of Excess Iron and Sources of Serum Ferritin in Patients with Thalassaemia

Abstract

Liver and spleen iron concentrations, serum ferritin level and binding of S‐ferritin to concanavalin A (Con A) were measured in 12 patients with thalassaemia major or intermedia at the time of splenectomy. All these subjects had increased liver iron concentration, most of them had hepatic fibrosis but none of them had histological evidence of chronic hepatitis. No patient had ascorbic acid deficiency. Serum ferritin concentration was increased in all cases, ranging from 266 to 5 504 μg/l. In all but 2 subjects most of the protein did not bind to Con A, thus behaving as tissue ferritin. There were highly significant correlations between serum ferritin concentration, amount of blood transfused and liver iron concentration. On the average, iron concentration in the liver was about 3 times that in the spleen. The findings obtained suggest that in patients with thalassaemia major or intermedia most of the iron is deposited in parenchymal tissues and most of the S‐ferritin derives by leakage from the cytosol of iron‐loaded parenchymal cells. S‐ferritin is a valid index of liver iron overload in thalassaemic patients without complications such as viral hepatitis and/or ascorbic acid deficiency.

Myelodysplastic Syndrome: An Egyptian Experience

“…Highly significant correlations between serum ferritin concentrations, amount of blood transfused and liver iron concentration has been documented in previous studies [24]. 25/30(83.3%) males were cigarette smokers.…”

The heart in transfusion dependent homozygous thalassaemia today – prediction, prevention and management

“…It has been shown that high levels of ferritin may be present even before the tissue iron storage is excessive. This may be caused by leakage across the cell membranes because of the iron toxicity (73). In contrast, intensified chelation therapy in heavily iron loaded patients, may rapidly reduce serum ferritin because the cell membranes are stabilised, while tissue iron, especially in the heart, remains elevated (74).…”

Normalized left ventricular volumes and function in thalassemia major patients with normal myocardial iron

“…It has been reported that only 50% of UK patients survive beyond the age of 35 years (3); however, more recent cohorts have shown improved survival (4). Both serum ferritin measurements (5–8) and liver biopsy estimations (9–11) have limitations in the assessment of myocardial iron, and recent reports have shown a poor correlation between myocardial T2* with hepatic T2* in a cross section of patients (12) and in patients receiving different chelation regimes (13).…”