Serum ferritin in children with thalassaemia regularly transfused

Abstract

SYNOPSIS A controlled trial of continuous chelation therapy in regularly transfused children with homozygous fl-thalassaemia has been in progress at the Hospital for Sick Children since April 1966. In the sixth and seventh years of the trial the effect of this treatment on iron overload has been assessed by estimating serum ferritin levels and liver iron concentrations in both chelator-treated and control groups. When compared with non-chelated controls, results of both these estimations were invariably lower in the chelated group. However, all the results in both groups were very high, and fell within the ranges observed in untreated idiopathic haemochromatosis. A close correlation was found between serum ferritin levels and liver iron concentrations in these children, indicating that serum ferritin is a valuable alternative to liver iron concentration in the assessment of visceral iron overload, even when massive tissue siderosis is present.Without blood transfusion the majority of patients with homozygous P-thalassaemia die from anaemia in early childhood. With regular transfusions, however, survival is prolonged into adolescence or early adult life (Weatherall and Clegg, 1972). The major problem in management, and the chief factor limiting life in these patients, then becomes that of iron overload, the excess iron being derived predominantly from transfused red blood cells.Parenteral chelating agents have been used in thalassaemia in an attempt to promote mobilization and excretion of the excess iron but at present there is little information regarding their real value in this condition. A prospective trial of long-term chelation therapy in regularly transfused children with homozygous thalassaemia was started at the Hospital for Sick Children in 1966. During the sixth and seventh years of the trial serum ferritin levels were measured in both chelator-treated patients and in untreated controls. The results of these estimations have been compared with liver iron concentrations in the same patients, and serum ferritin levels have also been determined in other regularly transfused thalassaemic children not included in the trial. The results obtained provide information regarding the effects of chelation therapy and the value of the serum ferritin level as a measure of iron overload in thalassaemia.

Italian Society of Hematology practice guidelines for the management of iron overload in thalassemia major and related disorders

“…There was good linear correlation (R 2 >0.5) in four out of the remaining studies 17,18,23,25 but in one of them, only in patients with lower iron burden. 18 Quality limitation of the studies were sample size (only four studies had analyzed more than 100 patients), and directness (all of them analyzed a population of patients who had started iron chelation therapy and included patients with high iron burden).…”

“…4,9,[18][19][20][21][22][23][24] Strong linear correlation (R 2 =approximately 1.0), enabling precise quantitative estimation of body iron content, was demonstrated in one study 20 but only in patients without a histological picture of liver hepatitis. There was good linear correlation (R 2 >0.5) in four out of the remaining studies 17,18,23,25 but in one of them, only in patients with lower iron burden. 18 Quality limitation of the studies were sample size (only four studies had analyzed more than 100 patients), and directness (all of them analyzed a population of patients who had started iron chelation therapy and included patients with high iron burden).…”

ß-Thalassemia Major: Experience at King Fahad Hofuf Hospital, Al-Hassa, Saudi Arabia

“…4,15 Although it is best to treat ß-thalassemia major by regular and aggressive blood transfusion so as to promote normal physical growth, avoid skeletal deformity and control morbidity from hypoxia, 15 such an aggressive transfusion program is unfortunately often complicated by iron overload, especially if not accompanied by adequate iron chelation. 16 We could not undertake such a hypertransfusion regime because of noncompliance of our patients with DF infusion. But despite the fact that our patients were undertransfused, they still had high serum ferritin levels.…”

Correlation of myocardial performance index and serum ferritin levels in children with beta thalassemia major

“…This disparity could go in lines with recent studies that showed ferritin level was not correlated well with cardiac iron. [20][21][22] In current study rate of increase of MPI, IVCT, IVRT and ET was observed to be higher up to SF of 1962µg/L, thereafter the change was slower. This observation in current study is consistent with observation made by A. Shahmohammadi et al in which there was no significant change in MPI for SF levels between less than 2000 µg/L and more 2000 µg/L.…”