Journal Article10.1097/MOP.0B013E328321882E
Current issues in blood transfusion for sickle cell disease.
Shannon Kelly Wahl,Keith Quirolo +1 more
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TL;DR: Red cell transfusion is currently the most studied and accepted therapy for most acute and many chronic complications of SCD and Physicians caring for patients with sickle cell disease should be aware of the unique complications and transfusion requirements in this population.
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Abstract: Purpose of review Although blood transfusion has been felt to be a beneficial therapy for sickle cell disease (SCD) since the 1950s, associated complications initially limited this therapy for these patients. With advances now reducing the side effects of transfusion and several landmark studies over the last decade clearly defining the efficacy for decreasing sickle cell morbidity, the indications for transfusion have increased. This review will discuss the indications, methods and goals of transfusion as well as complications and recent changes in transfusion therapy for SCD. Recent findings Recently studies have established the efficacy of transfusion for prevention of stroke, treatment of acute chest syndrome and perioperative transfusion management of SCD. Pulmonary hypertension is increasingly recognized as a significant source of morbidity and mortality and is an evolving indication for transfusion therapy. Phenotypically matching transfused blood has been shown to decrease alloimmunization, and genotyping for antigen matching may help match donors to patients in the future. Summary The increased use of transfusions may ultimately be balanced by hydroxyurea and other newer therapies developed as the complex pathophysiology of SCD is better understood; however, red cell transfusion is currently the most studied and accepted therapy for most acute and many chronic complications of SCD. Physicians caring for patients with sickle cell disease should be aware of the unique complications and transfusion requirements in this population.
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Citations
•Journal Article
Red Blood Cell Alloimmunization among Sudanese Homozygous Sickle Cell Disease Patients
TL;DR: Although limited preco mpatibility testing is done in blood banking services in Sudanese hospital, low incidence of red cell allo immun izat ion (4%) was observed in this study and it is recommended that to improve the blood bank services and polices to overcome the risk of allo Immunization.
Optimal Manual Exchange Transfusion Protocol for Sickle Cell Disease: A Retrospective Comparison of Two Comprehensive Care Centers in the United Kingdom and Canada.
TL;DR: A retrospective cohort study of two different manual RBC exchange transfusion methods was conducted between two comprehensive care centers in Toronto, ON, Canada and London, United Kingdom, finding that the unit-based method was more effective at maintaining the prescribed Hb S target.
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Comparing abstract numerical and visual depictions of risk in survey of parental assessment of risk in sickle cell hydroxyurea treatment.
Chavis A. Patterson,Lamia P. Barakat,Phyllis K. Henderson,Faith Nall,Anna Westin,Carlton Dampier,Lewis L. Hsu +6 more
TL;DR: Given earlier concerns about poor “numeracy” in the US population, visual depictions of risk could be an effective tool for routine communication in health education and medical decision-making.
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A Targeted Approach to Increasing the African American Blood Donor Pool
Arnethea L Sutton
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TL;DR: A targeted educational approach, incorporating the Theory of Planned Behavior and various teaching methods, to motivate African Americans non-donors to attempt to donate blood resulted in a statistically significantly higher proportion of African Americans presenting to donate than the current proportion in Virginia.
A Young Adult Jehovah’s Witness With Severe Anemia
TL;DR: Two of the most ethically complex situations in pediatrics are those involving families whose religious beliefs preclude the provision of life-sustaining treatment and those involving young adults who have reached the age of legal majority and who face decisions about life-saving treatment.
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TL;DR: Transfusion greatly reduces the risk of a first stroke in children with sickle cell anemia who have abnormal results on transcranial Doppler ultrasonography.
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Prevention of a first stroke by transfusions in children with sickle cell anemia and abnormal results on transcranial doppler ultrasonography
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Elliott Vichinsky,Lynne Neumayr,A. Earles,Roger Williams,Evelyne T. Lennette,Deborah Dean,Bruce G. Nickerson,Eugene P. Orringer,Virgil McKie,Rita Bellevue,Charles Daeschner,E A Manci +11 more
TL;DR: A treatment protocol that included matched transfusions, bronchodilators, and bronchoscopy was evaluated and it was found that infection and fat embolism are underdiagnosed in patients with the syndrome.
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TL;DR: A wealth of information is produced on the mechanisms by which a single base substitution in the gene encoding the human β-globin subunit, with the resulting replacement of β6 glutamic acid by valine, leads to the protean and devastating clinical manifestations of sickle cell disease.
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Mark A. Tanner,Renzo Galanello,Carlo Dessì,Gillian C. Smith,Mark Westwood,Annalisa Agus,M Roughton,Ravi Assomull,Sunil V. Nair,John Walker,Dudley J. Pennell +10 more
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