title: Transfusion therapy for sickle cell disease: a balancing act.
abstract: Transfusion therapy is a key intervention in decreasing morbidity and mortality in patients with sickle cell disease (SCD). Current indications for acute and chronic transfusion therapy have significantly increased the number of RBC units transfused to patients with SCD worldwide. This review summarizes transfusion management for the treatment or prevention of neurologic and perioperative complications, acute chest syndrome, and acute anemia associated with SCD. Despite the recognized benefits of transfusion therapy, it is not without the risks of iron overload, alloimmunization, and delayed hemolytic transfusion reactions. Transfusional iron overload management includes automated RBC exchange, noninvasive imaging to monitor iron burden, and iron chelation with parenteral or oral agents. Although limited and extended RBC antigen matching reduces antibody formation, the prevalence of RBC alloimmunization in patients with SCD remains high. Recent studies demonstrate that RH genetic diversity in patients with SCD contributes to Rh alloimmunization, suggesting that even more refined RBC matching strategies are needed. Advances in molecular blood group typing offer new opportunities to improve RBC matching of donors and recipients and can be of particular benefit to patients with SCD.
authors: 1Division of Hematology, The Children's Hospital of Philadelphia, Philadelphia, PA.
authors: Stella T
authors: ST
authors: Chou
mesh_terms: D056586:Acute Chest Syndrome / Q000209:etiology / Q000517:prevention & control; D000755:Anemia, Sickle Cell / Q000628:therapy*; D001782:Blood Donors; D001787:Blood Group Incompatibility / Q000209:etiology / Q000517:prevention & control; D001788:Blood Grouping and Crossmatching; D017707:Erythrocyte Transfusion / Q000009:adverse effects* / Q000379:methods*; D006461:Hemolysis / Q000187:drug effects; D006801:Humans; D007502:Iron Chelating Agents / Q000627:therapeutic use; D019190:Iron Overload / Q000209:etiology / Q000517:prevention & control; D009422:Nervous System Diseases / Q000209:etiology / Q000517:prevention & control; D012204:Rh-Hr Blood-Group System; D012307:Risk Factors
chemical_list: D007502:Iron Chelating Agents; D012204:Rh-Hr Blood-Group System