Benefits of Transfusion in Haemoglobinopathies - Sickle Cell Disease (SCD) and Thalassemia


Sickle Cell Disease (SCD) and Thalassemia are complex hemoglobinopathies with distinct clinical manifestations and treatment requirements. In managing patients with these conditions, transfusion emerges as a cornerstone of care, offering numerous life-saving benefits. 

For SCD patients, transfusion serves both acute and long-term needs. Acute indications encompass critical situations where transfusion can be life-saving, as well as regular therapy for ongoing management. In cases where evidence is limited, transfusion decisions should be made on a case-by-case basis, considering the patient’s unique circumstances. 

In patients with Homozygous ß Thalassemia, transfusion needs are categorized as “Transfusion-Dependent Thalassemia (TDT)” and “Non-Transfusion-Dependent Thalassemia (NTDT).” TDT patients require regular transfusions from infancy to manage severe anemia and suppress ineffective erythropoiesis. NTDT patients may not require regular transfusions but might need them under specific conditions, such as worsening anemia due to physiological stressors. 

A comprehensive guideline for transfusing patients with SCD and thalassemia exists, but individual patient considerations should guide clinical decisions. Pre-transfusion testing, including extended RBC phenotype and antibody screening, is crucial for patient safety and compatibility. Phenotype-matched components should be provided based on established guidelines. Furthermore, fresh RBC units are preferred, and serologic testing for hepatitis and cytomegalovirus status is essential. 

Chronic transfusions in SCD are associated with iron overload risks, necessitating regular monitoring and chelation therapy. Transfusion in SCD patients proves beneficial in various acute scenarios, including ischemic stroke, acute anemia, acute chest syndrome, and severe sepsis, among others. For thalassemia patients, regular transfusions are initiated based on specific criteria, and close monitoring is vital to assess their transfusion requirements. Chronic transfusion is recommended for primary and secondary stroke prevention in SCD patients, and prophylactic transfusion may be considered for pregnant patients and those with multiple pregnancies. 

In homozygous ß Thalassemia, TDT patients benefit from regular transfusions initiated during infancy to manage severe anemia and other related complications. For NTDT patients, decisions for transfusions should consider factors beyond Hb levels alone, with an emphasis on patient needs and potential risks.