Introduction: Hyperhemolysis is a life-threatening condition of
exaggerated hemolysis of red blood cells which occurs in patients
receiving chronic transfusion therapy. Results: We present a 19-year-old
male with β-thalassemia major with an episode of hyperhemolysis.
Hemolysis was initially unresponsive to immunosuppression, but responded
to with the addition of eculizumab. Several weeks after stabilization,
hemolysis returned which was also successfully managed with
immunosuppression and eculizumab. Discussion: Hyperhemolysis is unique
in β-thalassemia due to the underlying dysfunctional erythropoiesis and
transfusion dependence. Immunosuppression with eculizumab successfully
slowed the hemolysis and allowed for resumption of transfusions.