Hyperhaemolytic transfusion reaction in two β-thalassaemia major patients: The role of eculizumab.

What Is Known And Objective: Hyperhaemolytic transfusion reactions are rare life-threatening events predominantly affecting patients with haemoglobinopathies. We report two cases in β-thalassaemia major patients on chronic transfusion therapy and highlight the role of eculizumab in its management.

Case Summary: Patient 1 presented with intravascular haemolysis on day 7 (D7) post-transfusion and responded to treatment with corticosteroids and intravenous immunoglobulin. However, patient 2 presented with severe symptomatic anaemia (D4 post-transfusion) unresponsive to the aforementioned measures. Eculizumab administration led to resolution of the hyperhaemolysis.

What Is New And Conclusion: We report the successful management of hyperhaemolysis with eculizumab in a β-thalassemia major patient.