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.