Article

Blood transfusion usage among adults with sickle cell disease - a single institution experience over ten years

Molecular Haematology, Division of Gene and Cell Based Therapy, King's College London School of Medicine, Denmark Hill, London, UK.
British Journal of Haematology (Impact Factor: 4.96). 03/2011; 152(6):766-70. DOI: 10.1111/j.1365-2141.2010.08451.x
Source: PubMed

ABSTRACT Transfusion of red blood cells is a major therapeutic option in sickle cell disease (SCD). There is strong evidence for its efficacy, particularly in primary and secondary stroke prevention in children, however, its use in other areas remains controversial. This study assessed the patterns of transfusion in the adult cohort attending King's College Hospital over a 10-year period, from 2000 to 2009. Total blood usage has increased significantly (P = 0·006) during this time, with 78% of the blood received by only 6% of the patients. The increase is explained by increased automated red cell exchange and increased usage for planned and acute transfusions for sickle-related complications.

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    • "Blood transfusion is increasingly used in the management of SCD. Indeed, a recent audit of patients at King's College Hospital (London, UK) showed expanding indications , and a large number of patients presenting with clinically significant iron overload after a period of sporadic blood transfusions (Drasar et al, 2011). With repeated blood transfusion, iron deposits in target organs causing oxidative damage and fibrosis (Andrews, 2000). "
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    ABSTRACT: Spin density projection-assisted R2-magnetic resonance imaging (R2-MRI; FerriScan(®)) scans from 40 chelation-naïve sickle cell patients were used to assess renal iron load by measuring renal R2 (R-R2). Clinical data were collected retrospectively for the 2-year period preceding the scan. R-R2 showed no significant correlation with transfusional iron load (assessed by liver iron concentration), but correlated significantly with serum bilirubin (R = 0·61, P < 0·0001) and lactate dehydrogenase (R = 0·58, P < 0·0001). Mean (±standard deviation) R-R2 was higher (P = 0·02) in patients with renal hyperfiltration (29·8 ± 10·3/s) than those without (23·11 ± 6·6/s). Five patients had significantly lower signal intensity in the renal cortex, as compared to the medulla. These patients had a significantly higher (P < 0·0001) mean R-R2 than those showing no cortico-medullary difference. We postulate that the increased R-R2 is associated with haemolysis rather than transfusional iron load in sickle cell disease.
    British Journal of Haematology 03/2012; 157(5):599-605. DOI:10.1111/j.1365-2141.2012.09093.x · 4.96 Impact Factor
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    • "Blood transfusion therapy usage in sickle cell disease (SCD) is increasing (Drasar et al, 2011) and with this comes the potential for morbidity and mortality associated with the consequent iron overload. Whilst patients treated with regular blood transfusions have their iron indices monitored regularly and are under regular clinical review, patients receiving sporadic blood transfusions can accrue a substantial iron load relatively unnoticed. "
    British Journal of Haematology 02/2012; 157(5):645-7. DOI:10.1111/j.1365-2141.2012.09060.x · 4.96 Impact Factor
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    • "Moreover , even when the number of units was reported , the units are often listed as a standard 250 or 300 ml , so the total amount of infused iron is unknown . Finally , the increased use during the last 10 years of automated red cell exchange should have fewer body iron burden implications ( Drasar et al , 2011 ) . Fung et al ( 2007 ) suggested that hospi - talization in SCD is not related to iron overload as assessed by serum ferritin levels or LIC . "
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    ABSTRACT: Blood transfusions may prevent and treat serious complications related to sickle-cell disease (SCD) when performed according to specific guidelines. However, blood transfusion requirements in SCD inevitably lead to increased body iron burden. An adequate chelation treatment may prevent complications and reduce morbidity and mortality. This review evaluates the effectiveness, safety and costs of chelation treatment. The included trials were examined according to the recommendations of the American College of Cardiology (ACC) and the American Heart Association (AHA). Overall, 14 trials and a total of 502 patients with SCD were included in this review. Deferoxamine alone (s.c. or i.v.), deferiprone alone or versus deferoxamine, deferasirox versus deferoxamine and combined treatment with deferoxamine plus deferiprone were included and evaluated in the analysis. Only two randomized clinical trials have been reported. The results of this analysis suggest that use of chelation treatment in SCD to date has been based on little efficacy and safety evidence, although it is widely recommended and practised. The cost/benefit ratio has not been fully explored. Further research with larger randomized clinical trials needs to be performed.
    British Journal of Haematology 06/2011; 154(5):545-55. DOI:10.1111/j.1365-2141.2011.08769.x · 4.96 Impact Factor
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