Post-transfusion hyperhemolysis syndrome in a patient with beta thalassemia major

Hyperhemolysis syndrome (HS) is characterized by the occurrence of severe anemia with post-transfusion hemoglobin and hematocrit levels being markedly lower than those present prior to transfusion. A high index of suspicion of HS in a multi-transfused thalassemia patient allows prompt institution of therapy resulting in improved survival outcome.