Journal
HEMATOLOGY-AMERICAN SOCIETY OF HEMATOLOGY EDUCATION PROGRAM
Volume -, Issue 1, Pages 160-166Publisher
AMER SOC HEMATOLOGY
DOI: 10.1182/hematology.2020000102
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The introduction of regular red cell transfusions 60 years ago transformed beta-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 beta 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.
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