Exchange blood transfusion compared with simple transfusion for first overt stroke is associated with a lower risk of subsequent stroke: A retrospective cohort study of 137 children with sickle cell anemia

The Children's Hospital of Philadelphia, Filadelfia, Pennsylvania, United States
Journal of Pediatrics (Impact Factor: 3.79). 12/2006; 149(5):710-2. DOI: 10.1016/j.jpeds.2006.06.037
Source: PubMed


A retrospective cohort study of children with sickle cell anemia (SCA) and strokes was used to test the hypothesis that exchange transfusion at the time of stroke presentation more effectively prevents second strokes than does simple transfusion. Children receiving simple transfusion had a 5-fold greater relative risk (95% confidence interval = 1.3 to 18.6) of second stroke than those receiving exchange transfusion.

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Available from: Michael R DeBaun, Jun 11, 2014
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    • "Recurrent silent infarcts, despite transfusion therapy, also lead to further cognitive deficits [25]. The devastating outcomes of acute stroke in patients with SCD demand further study, not only to define additional risk factors, but also to identify successful interventions [29] [30] [31] [32]. Although further research is needed, the positive outcome of our patient with hemoglobin SC suggests that identifying PFOs and closing them nonsurgically could be beneficial in selected patients. "
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    ABSTRACT: Although individuals with sickle cell disease (SCD) are at increased risk for stroke, the underlying pathophysiology is incompletely understood. Intracardiac shunting via a patent foramen ovale (PFO) is associated with cryptogenic stroke in individuals without SCD. Recent evidence suggests that PFOs are associated with stroke in children with SCD, although the role of PFOs in adults with stroke and SCD is unknown. Here, we report 2 young adults with SCD, stroke, and PFOs. The first patient had hemoglobin SC and presented with a transient ischemic attack and a subsequent ischemic stroke. There was no evidence of cerebral vascular disease on imaging studies and the PFO was closed. The second patient had hemoglobin SS and two acute ischemic strokes. She had cerebral vascular disease with moyamoya in addition to a peripheral deep venous thrombosis (DVT). Chronic transfusion therapy was recommended, and the DVT was managed with warfarin. The PFO was not closed, and the patients' neurologic symptoms were stabilized. We review the literature on PFOs and stroke in SCD. Our cases and the literature review illustrate the dire need for further research to evaluate PFO as a potential risk factor for stroke in adults with SCD.
    07/2013; 2013(1):516705. DOI:10.1155/2013/516705
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    • "While this makes theoretical sense in attempting to reduce iron burden, the degree of practical benefit from this procedure has not been rigorously demonstrated to date. In the setting of acute stroke, a retrospective study has suggested that exchange transfusion is associated with a reduced risk of recurrence of stroke long-term(Hulbert, et al 2006). As this is a retrospective study, it cannot be considered definitive; however, at this point, these data suggest that exchange transfusion or erythrocytapheresis should be considered the treatment of choice for acute stroke in SCA when feasible. "
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    ABSTRACT: This review will focus on the strengths and limitations associated with the current standard of care for primary prevention of ischaemic strokes in children with sickle cell anaemia (SCA) - transcranial Doppler ultrasound (TCD) screening followed by regular blood transfusion therapy when TCD measurement is above a threshold defined by a randomized clinical trial (RCT). The theoretical basis for potential alternative strategies for primary prevention of neurological injury in SCA is also discussed. These strategies will include, but will not be limited to: immunizations to prevent bacterial infections, particularly in low income countries; management of elevated blood pressure; and targeted strategies to increase baseline haemoglobin levels with therapies such as hyroxycarbamide or potentially definitive haematopoietic stem cell transplant.
    British Journal of Haematology 01/2012; 157(1). DOI:10.1111/j.1365-2141.2011.09005.x · 4.71 Impact Factor
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    • "In the only prospective Figure 2. Increased use of exchange blood transfusion therapy as the predominant method of transfusion therapy compared with simple transfusion therapy for the acute presentation of overt stroke in 14 pediatric hematology centers. (Reprinted with permission from Hulbert et al, 2006. 19 ) "
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    ABSTRACT: Overt strokes, previously one of the most common neurological complications in sickle cell disease (SCD), have become far less frequent with routine transcranial Doppler (TCD) assessment followed by regular blood transfusion therapy. Nevertheless, children and adults with SCD continue to have overt strokes, and in the foreseeable future will continue to require secondary prevention of strokes. With the exception of the most recently completed "Stroke With Transfusions Changing to Hydroxyurea" Trial (SWiTCH; NCT00122980), randomized trials providing best evidence for long-term management of overt strokes in SCD is lacking. Instead of randomized clinical trials, a series of observational and single-arm studies have predominated. This review assesses the best available evidence for acute and chronic management of overt stroke and the efficacy of regular blood transfusion therapy, hydroxyurea therapy, and hematopoietic stem cell transplantation (HSCT), including matched sibling donor and unrelated HSCT.
    Hematology 12/2011; 2011(1):427-33. DOI:10.1182/asheducation-2011.1.427 · 0.81 Impact Factor
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