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: 4.71). 03/2011; 152(6):766-70. DOI: 10.1111/j.1365-2141.2010.08451.x
Source: PubMed


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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Available from: Alex Mijovic, Oct 06, 2014
    • "). The beneficial effects of transfusion therapy observed in recent clinical trials are expanding their indication and utilization, contributing to an increased use of blood (Drasar et al, 2011; Steinberg, 2014). Nonetheless, transfusion is not without risks; haemolytic transfusion reactions and iron overload are common side effects, while transmission of infections remains a significant issue in some countries. "
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    ABSTRACT: Transfusion therapy is effective in the prevention and treatment of many complications of sickle cell disease (SCD). However, its benefits must be balanced against its risks, including delayed haemolytic transfusion reactions (DHTR). Not only is the relative rate of alloimmunization higher in patients with SCD than in other patient populations, but attendant risks associated with DHTR are even greater in SCD. Clinicians' awareness of DHTR events is poor because symptoms of DHTR mimic acute vaso-occlusive pain and immunohaematology findings are often negative. Transfusions delivered in the acute rather than elective setting appear to confer a higher risk of DHTR. Management of DHTR in SCD depends on the clinical severity, ranging from supportive care to immunosuppression, and optimization of erythropoiesis. DHTR must be considered in any recently transfused patient presenting with acute sickle cell pain. Meticulous documentation of transfusion and immunohaematology history is key. We anticipate an increase in DHTR events in SCD patients with the increasing use of red blood cell transfusion therapy. © 2015 John Wiley & Sons Ltd.
    British Journal of Haematology 05/2015; 170(6). DOI:10.1111/bjh.13494 · 4.71 Impact Factor
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    • "We chose to study the efficacy of transfusion to treat VOE as it is known to acutely reverse clinical symptoms in other vaso-occlusive manifestations of SCD, such as ACS and stroke (Mallouh & Asha, 1988; Emre et al, 1995; Vichinsky et al, 2000), and medications designed to target the underlying VOE pathology, such as poloxamer 188 (Gibbs & Hagemann , 2004) and inhaled nitric oxide (Gladwin et al, 2011) have failed to show benefit in clinical trials. Although virtually all published SCD transfusion guidelines do not consider VOE an indication for transfusion, patients are frequently transfused during VOE in clinical practice (Drasar et al, 2011; Raphael et al, 2013). "

    British Journal of Haematology 04/2015; 171(2). DOI:10.1111/bjh.13390 · 4.71 Impact Factor
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    • "Blood transfusion is increasingly used in the prevention and treatment of many of the complications of sickle cell disease (SCD) (Drasar et al, 2011). The benefits of transfusion must be weighed against its risks, including iron overload, infections and haemolytic transfusion reactions. "
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    ABSTRACT: Delayed haemolytic transfusion reactions (DHTR) are potentially life-threatening complications in patients with sickle cell disease (SCD). Between 1 August 2008 and 31 December 2013, 220 of 637 adult patients in our centre had at least one red blood cell (RBC) transfusion in 2158 separate transfusion episodes. Twenty-three DHTR events occurred in 17 patients (13 female) including 15 HbSS, one HbSC and one HbSβ(0) thalassaemia, equating to a DHTR rate of 7·7% of patients transfused. Mean interval from RBC transfusion to DHTR event was 10·1 ± 5·4 d, and typical presenting features were fever, pain and haemoglobinuria. Twenty of the 23 (87·0%) DHTR episodes occurred following transfusion in the acute setting. Notably, 11/23 (47·8%) of DHTRs were not diagnosed at the time of the event, most were misdiagnosed as a vaso-occlusive crisis. 16/23 DHTRs had 'relative reticulocytopenia', which was more common in older patients. Seven of 23 episodes resulted in alloantibody formation, and three caused autoantibody formation. DHTRs are a severe but uncommon complication of RBC transfusion in SCD and remain poorly recognized, possibly because they mimic an acute painful crisis. Most of the DHTRs are triggered by RBC transfusion in the acute setting when patients are in an inflammatory state. © 2015 John Wiley & Sons Ltd.
    British Journal of Haematology 03/2015; 169(5). DOI:10.1111/bjh.13339 · 4.71 Impact Factor
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