Challenges in chronic transfusion for patients with thalassemia

Abstract

The introduction of regular red cell transfusions 60 years ago transformed β-thalassemia major from a fatal childhood illness into a chronic disorder. Further advances in the prevention of transfusion-transmitted infections and management of iron overload have allowed survival and quality of life to approach normal. However, transfusion therapy for some other thalassemia syndromes continues to challenge clinical decision-making. Nearly one-half of the patients with E ß thalassemia are transfusion-dependent, yet the criteria for initiating transfusions or hemoglobin targets are not well defined. Patients with thalassemia intermedia who begin transfusions as adults are at very high risk for developing red cell alloimmunization and serious hemolytic transfusion reactions. In the growing number of survivors of Bart hydrops fetalis, the approach to transfusion therapy and iron chelation is rapidly evolving. A collaboration between hematology and transfusion medicine specialists will be essential to improving patient care and developing evidence-based guidelines.

Non‐transfusion dependent thalassemia is independently associated with higher alloimmunization risk than transfusion dependent thalassemia and would benefit the most from extended red cell antigen‐matching

“…These alloimmunized NTDT patients are therefore more likely to receive RBCs expressing the corresponding antigen and mount an anamnestic alloantibody response, resulting in hemolytic transfusion reactions. 15 This further supports the importance of RBC antigen-matching for the NTDT patients, even in the absence of detectable alloantibodies.…”

Deformability of Stored Red Blood Cells

“…In particular, transfused RBCs from some units can function for long periods, while others are rapidly eliminated from the recipient's bloodstream (Bosman, 2013;Dinkla et al, 2014;Tzounakas et al, 2016;Roussel et al, 2018). This situation is highly undesirable for chronic transfusion recipients because of potential adverse effects, such as iron overload (Lal, 2020). Therefore, there is a need to develop reliable biomarkers to attest to the quality of PRBCs and to optimize the management of units stored in the blood bank.…”

The transfusion management of beta thalassemia in the United States

“…Historically, the broad categories of ethnicities were Mediterranean and Asian (Southeast Asia and China), but to these should also be added South Asian and the Middle Eastern populations 6 . There are significant proportions of patients with HbE β thalassemia and α thalassemia, where the approach to chronic transfusions is distinct from β thalassemia major 59–61 . The red cell antigen disparity between donors and recipients is considerably greater due to differences in ethnicity, which amplifies the risk of alloimmunization 9,62,63 .…”