Sickle cell disease (SCD) and thalassemia are complex hemoglobinopathies. Although they are grouped here together, their clinical manifestations and treatment modalities are different. Red blood cell (RBC) transfusion is a cornerstone for the management of patients with SCD and thalassemia. Transfusion in SCD patients can be for acute indications where transfusion can be life-saving, or for regular long-term therapy. These indications range from those in which transfusion is strongly recommended to those where it is unproven or controversial, and therefore requiring individualized decision. There are insufficient randomized clinical trials (RCT) to direct the clinicians on when to transfuse SCD patients. Transfusion for indications where evidence is limited should be based on a case-by-case assessment.
Homozygeous ß Thalassemia can be broadly clinically categorized as
“transfusion-dependent thalassemia, TDT” who require regular transfusion from infancy to sustain life and suppress ineffective erythropoiesis, and
“non-transfusion dependent thalassemia, NTDT” who have moderate hemolytic anemia but maintaining a hemoglobin (Hb) level that is sufficient for growth and development without transfusion support.(1)
However, these patients may have worsening anemia particularly upon exposure to physiological stressor or other triggers which necessitates transfusion support. (2)