Transfusional siderosis: The effects of excessive iron deposits on the tissues

Cappell, D. F.; Hutchison, H. E.; Jowett, M.

doi:10.1002/path.1700740202

Cited by 68 publications

(34 citation statements)

References 20 publications

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“…An alternative suggestion is that the decrease in storage iron relative to the transfused load observed in heavily ironloaded patients is due to the onset of spontaneous iron excretion. This view is supported by a number of reports (Howell and Wyatt, 1953;Cappell, Hutchison, and Jowett, 1957;Witzleben and Wyatt, 1961) indicating that estimates of cadaver iron in heavily transfused subjects fall far short of the total amount of iron received in life.…”

Section: Discussionsupporting

confidence: 62%

Serum ferritin in children with thalassaemia regularly transfused

Letsky¹,

Miller²,

Worwood³

et al. 1974

J Clin Pathol

102

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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.

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Section: Discussionsupporting

confidence: 62%

Serum ferritin in children with thalassaemia regularly transfused

Letsky¹,

Miller²,

Worwood³

et al. 1974

J Clin Pathol

102

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“…Given the inability to measure pulmonary iron concentration in vivo, the extent to which this assumption is valid may be open to criticism. However, necropsy data in TM patients with transfusional siderosis show that the iron content in the lung is generally a fixed proportion of that in the liver [6,7]. The present T2 relaxation times represent mean values of five measurements obtained in the periphery of the liver.…”

Section: Discussionmentioning

confidence: 83%

“…Furthermore, a cause-and-effect relationship of pulmonary iron deposition and restrictive abnormalities in TM is not supported by necropsy data reported in patients with siderosis. In these studies, iron was found predominantly in bronchial epithelial cells and bronchial glands rather than in the lung parenchyma [6,7].…”

Section: Discussionmentioning

confidence: 89%

Respiratory function in patients with thalassaemia and iron overload

Dimopoulou¹,

Kremastinos²,

Maris³

et al. 1999

Eur Respir J

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Iron deposition in the respiratory system has been proposed as a potential cause of the ventilatory restrictive impairment seen in patients with thalassaemia major (TM) and iron overload. In this study, magnetic resonance imaging (MRI) measurements of the liver (T2 relaxation time) were used as a surrogate index of total body iron burden and the extent to which these measurements correlated with total lung capacity (TLC) in patients with TM was examined.Twenty-one patients (aged 255 yrs) with TM participated in the study. Standard pulmonary function tests were undertaken and the T2 relaxation time of the liver was measured in all patients.Ventilatory restrictive impairment (mean TLC 7411 (SD)% predicted) was the most common abnormality found in 71% of TM patients. There was no correlation between TLC (% pred) and T2 relaxation time (r=0.06, p=0.78). T2 relaxation time correlated weakly with average serum ferritin levels (r=-0.56, p=0.008).In conclusion, the data do not support the notion that the restrictive impairment in patients with thalassaemia major and iron overload is related to iron deposition in the respiratory system. Eur Respir J 1999; 13: 602±605.

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“…However, this may result in generalized iron loading, such as in the heart, liver, endocrine organs and lungs [2]. Iron deposition in the lungs has been observed on postmortem examination of patients receiving multiple blood transfusions [3,4]. Pulmonary iron deposition may result in parenchymal damage.…”

mentioning

confidence: 99%

Lungs in thalassaemia major patients receiving regular transfusion

Tai¹,

Wang²,

Lou³

et al. 1996

European Respiratory Journal

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Progressive tissue iron deposition from multiple blood transfusions is common in beta-thalassaemia and pulmonary iron deposition may result in parenchymal damage. The objectives of this study were to: 1) determine the predominant pulmonary dysfunction in patients with thalassaemia major; and 2) demonstrate that parenchymal disease, if present, is at the level of the alveolocapillary membrane.Fourteen thalassaemia major patients (13 nonsmokers) receiving regular blood transfusion and without any history of chronic respiratory disease were recruited. Pulmonary function tests and echocardiography were performed before the scheduled transfusions. Three patients with the most restricted lung function were selected for high resolution computerized tomography (CT) of the lungs.One patient had an obstructive pattern with a forced expiratory volume in one second as percentage of forced vital capacity (FEV1/FVC) of 71%. Four patients demonstrated a restrictive pattern, as defined by total lung capacity (TLC) less than 80% predicted with normal FEV1/FVC%. Twelve patients had pulmonary transfer factors for carbon monoxide (TL,CO) below 80% pred, even after correction for the anaemia, indicating parenchymal disease. Eight of these 12 patients had alveolocapillary membrane defect, as demonstrated by a gas transfer factor of the pulmonary membrane (Tm) less than 80% pred. Mean resting arterial oxygen saturation was 95±2 (range 92-98) %. Eleven patients had oxygen desaturation of 5% or more during exercise on a bicycle ergometer, consistent with interstitial lung disease. There was no clinical or echocardiographic evidence of heart failure. Percentage predicted TLC was inversely correlated with age (r=-0.547; p=0.043). Both percentage predicted TLC and TL,CO were not correlated with iron burden or desferoxamine ratio. High resolution CT in the three selected patients showed no evidence of pulmonary fibrosis. We conclude that thalassaemia major patients have a predominant restrictive lung dysfunction with pulmonary parenchymal disease and alveolocapillary membrane block. The restrictive and interstitial lung disease could not be accounted for by iron loading or pulmonary fibrosis in our patients.

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Transfusional siderosis: The effects of excessive iron deposits on the tissues

Cited by 68 publications

References 20 publications

Serum ferritin in children with thalassaemia regularly transfused

Serum ferritin in children with thalassaemia regularly transfused

Respiratory function in patients with thalassaemia and iron overload

Lungs in thalassaemia major patients receiving regular transfusion

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