The management of fatal hyperhemolysis in a sickle cell anemia patient transfused in a remote Island in North Andaman

Abstract

Hyperhemolysis following red cell transfusion is a potentially fatal complication in sickle cell disease patients. Red cell alloimmunization can lead to a delayed hemolytic reaction further leading to hyperhemolysis resulting in the destruction of patients' own red blood cells. The chances of alloimmunization increases when there are ethnic differences between the donor and recipients. We report the case of a 25-year-old female with sickle cell anemia, treated with packed red cells in a remote island of North Andaman, India, who suffered from hemolytic transfusion reaction and hyperhemolysis yet could be successfully treated to recovery with the available resources. She had a severe hemolysis, anemia with the hemoglobin levels than pretransfusion hemoglobin, jaundice, acute kidney failure, and deranged liver function tests. Multiple alloantibodies “anti-C and anti-E” were found in her serum which were potentially hemolytic. She was treated with forced diuresis, steroids, and hemodialysis and recovered in a span of 10–14 days.