Hyperhemolysis syndrome in sickle cell disease: case report (recurrent episode) and literature review

Abstract

This is the second reported case of recurrent HS in a child. Recent studies have shown that the adhesion molecules expressed on RBC erythroid precursor cells and reticulocytes can interact with macrophages and can cause hemolysis. Because RBC alloantibodies and HLA antibodies were not identified in this case, it is believed that the patient's cells and transfused cells were destroyed by macrophages either by direct contact lysis or by erythrophagocytosis. The possible mechanism of IVIG and steroids on suppression of macrophages resulting in cessation of hemolysis is discussed. Our case illustrates the danger of recurrent HS and the difficulty of balancing this against the need for transfusions in patients presented with severe hemolysis.