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.

Prophylactic transfusion for pregnant women with sickle cell disease: a systematic review and meta-analysis

“…32,33 Although indiscriminate use of transfusion must be avoided, given its potential adverse effects of alloimmunization, acute and delayed transfusion reactions, transfusionrelated infectious morbidity, and iron overload, 13 transfusion in the appropriate context has merit and may even be lifesaving.…”

“…12 However, the use of prophylactic transfusion beyond these indications is tempered by concerns over acute and delayed transfusion reactions, alloimmunization, transfusion-related infections, and iron overload. 13 Furthermore, reports assessing the effect of prophylactic RBC transfusions on pregnancy outcomes in SCD have been inconsistent. [14][15][16][17][18][19] Thus, the objective of this study was to systematically review the impact of prophylactic transfusion compared with on-demand transfusion (defined as transfusion instituted to treat complications) on maternal and neonatal adverse pregnancy outcomes in pregnant women with SCD.…”

Factors Influencing RBC Alloimmunization: Lessons Learned from Murine Models

“…Ongoing studies are investigating which disease manifestations can be attributed solely to the altered β-globin gene and resultant RBC sickling, and which may be due to co-inheritance of immunoregulatory or other genes along with the sickle globin gene. It is well recognized that this patient population has amongst the highest levels of RBC alloimmunization following transfusion of any patient population [84,85,86]. However, there is much debate surrounding the reasons for the high rates of RBC alloimmunization [15,87,88], with potential factors including transfusion burden, RBC phenotypic differences between donors and recipients, and RBC genotypic variants in the sickle patients themselves.…”

Türkiye’ de orak hücre hastalığına sahip hastalarda eritrosit alloimmünizasyonu: tek merkez geriye dönük kohort çalışması

“…Repeated vasooclusive crisis can affect multiple organ systems, and individuals with SCD have increased risks of stroke, renal dysfunction, pulmonary hypertension, retinal disease, and avascular necrosis 9 . RBC transfusions remain a cornerstone treatment for acute and chronic complications in SCD 10 . Approximately 90% of adults with SCD will have received at least one RBC transfusion 9 .Transfusion therapy facilitates blood and tissue oxygenation and reduces the propensity for sickling by diluting the host cells and temporarily suppresses the production of HbS-containing red cells 11 .…”