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Publications

Journal Article Review Ppublish

PubMed: 40147455 DOI: 10.1111/bjh.20055

The wider perspective: Barriers and recommendations for transfusion support for patients with sickle cell disease in low- and middle-income countries.

Br J Haematol 206(6):1585-1592

Authors: Jeremy W Jacobs, Luiz Amorim, France Pirenne, Claude Tayou, Ijele Adimora, Lydia H Pecker, Aaron A R Tobian, Jeannie Callum, Julie Makani, Mark T Gladwin, Meghan Delaney, Darrell J Triulzi, Isaac Odame, Evan M Bloch

Affiliations

  • Department of Pathology, Microbiology, & Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.
  • HEMORIO, Instituto Estadual de Hematologia, Rio de Janeiro, Brazil.
  • Transfusion and Red Blood Cell Diseases, INSERM U955, The Mondor Institute for Biomedical Research, University Paris-Est Créteil, Paris, France.
  • Etablissement Français du Sang, International Affairs, Saint-Denis, France.
  • Haematology and Blood Transfusion Service, University Teaching Hospital, Yaoundé, Cameroon.
  • Division of Transfusion Medicine, Department of Pathology, Johns Hopkins Hospital, Baltimore, Maryland, USA.
  • Department of Medicine, Division of Hematology, Department of Gynecology & Obstetrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.
  • Department of Pathology and Molecular Medicine, Queen's University and Kingston Health Sciences Centre, Kingston, Ontario, Canada.
  • Department of Haematology and Blood Transfusion, Muhimbili University of Health and Allied Sciences (MUHAS), Dar-es-Salaam, Tanzania.
  • Sickle Cell Programme, Department of Haematology and Blood Transfusion, School of Medicine, MUHAS, Dar-es-Salaam, Tanzania.
  • Sickle Pan-African Consortium (SPARCO) Clinical Coordinating Centre, SickleInAfrica, Muhimbili University of Health and Allied Sciences (MUHAS), Dar-es-Salaam, Tanzania.
  • Division of Pulmonary and Critical Care Medicine, Department of Medicine, University of Maryland School of Medicine, Baltimore, Maryland, USA.
  • Division of Pathology & Laboratory Medicine, Children's National Hospital, Washington, District of Columbia, USA.
  • Department of Pathology, University of Pittsburgh, Pittsburgh, Pennsylvania, USA.
  • Division of Haematology/Oncology, Department of Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.

Abstract

Globally, sickle cell disease (SCD) is the most common inherited haemoglobinopathy. The highest burden of SCD is encountered in low- and middle-income countries (LMICs), most of which lack the resources to contend with the disease. There is a marked divide between care for individuals with SCD in high-income countries (HICs) versus LMICs, whereby the few disease-modifying therapies and curative regimens are only accessible to those in HICs. As such, blood transfusion remains central to the emergent treatment and prevention of complications of SCD. However, there are a myriad of related challenges in LMICs, which have impeded efforts to treat patients with SCD effectively. In addition to blood safety and availability, examples that impact SCD specifically include capabilities to detect and/or manage red blood cell alloimmunization, capacity for automated red cell exchange, limited immunohematology, suboptimal quality oversight with a lack of safeguards to prevent transfusion of incompatible blood and limited or absent post-transfusion surveillance to detect and/or manage transfusion-associated adverse events. Consequently, clinical practices that are otherwise regarded as standard of care in HICs remain the exception in LMICs, highlighting disparities in care. A multifaceted approach that prioritizes transfusion support in LMICs is needed to improve care for patients with SCD.

MeSH

Cells, Erythrocytes, Blood, Anemia, Sickle Cell, Income, Transfusion Medicine, Therapeutics

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