Sickle Hyper-hemolytic Transfusion Reaction Syndrome

Abstract

A 17-year-old female with sickle cell disease (SCD) presented with vasoocclusive crisis and jaundice, she received conventional treatment and one unit of compatible PRBC after a negative antibody screen was documented. After 48hrs of receiving a blood transfusion, her clinical picture was consistent with hemolysis. Immunohematology workup demonstrated evidence of a new antibody i.e anti-E. Delayed hemolytic transfusion reaction (DHTR) is a well-known complication of RBC transfusion. As her hemoglobin was declining , she was transfused with E antigen negative PRBCs. Despite this, hemolysis was ongoing and accelerated. Thus she was treated with steroids for presumed hyperhemolysis. Clinicians should have a high index of suspicion for hyperhemolysis in sickle cell patients with evidence of hemolysis after a recent transfusion. Differentiating hyperhemolysis from other hemolytic syndromes in SCD patient is critical; transfusions in a hyperhemolytic episode can accelerate hemolysis causing lifethreatening anemia.

Keywords

• delayed hemolytic transfusion reaction
• hyperhemolysis
• antibody screening
• red blood cell
• hemoglobin