Introduction
Hyperhemolysis is a rare, life-threatening condition, commonly occurring in the setting of chronic transfusion therapy. Hyper hemolysis results in hemolysis of both transfused and patient red blood cells.1 Delayed transfusion reactions, including hyperhemolysis reactions, have been shown to involve complement mediated hemolysis.2 Complement triggers the hemolytic reaction, and also amplifies the inflammatory response leading to enhanced tissue damage.3
A hyperhemolytic crisis is a grave situation with limited treatment options. Aggressive immunosuppression is often implemented to dampen the level of hemolysis. Eculizumab is a complement inhibitor currently approved for the treatment of complement mediated hemolysis in atypical hemolytic uremic syndrome (HUS) and paroxysmal nocturnal hemoglobinuria (PNH). There are several previously reported cases of eculizumab use in hyperhemolytic crises in sickle cell disease.4-9However, there is a paucity of evidence for eculizumab in transfusion-dependent β-thalassemia. Dysfunctional erythropoiesis and ineffective hemoglobin within red cells in β-thalassemia presents a unique challenge in the management of a hyperhemolytic crisis. We demonstrated effective management of acute and chronic hyperhemolysis in a patient β-thalassemia using eculizumab.