BACKGROUND Hyperhemolysis syndrome (HHS) is a severe delayed hemolytic transfusion reaction seen in sickle cell disease (SCD) patients, characterized by destruction of donor and recipient RBCs. It results in a drop in hemoglobin to below pretransfusion levels and frequently reticulocytopenia. CASE REPORT We report a case of a man in his 30s with SCD with a recent hospitalization two weeks prior for COVID-19. His red cell antibody history included anti-Fy(a) and warm autoantibody. At that time he was given two units of pRBC and discharged with a hemoglobin of 10.2 g/dL. He returned to the hospital approximately 1.5 weeks later with hemoglobin 6.0 g/dL and symptoms concerning for acute chest syndrome (ACS). Pretransfusion testing now showed 4+ pan-agglutinin in both gel-based and tube-based testing. Alloadsorption identified an anti-N and a strong cold agglutinin. Three least incompatible units were transfused to this patient over several days, with evidence of hemolysis. Further reference lab work revealed anti-Fya , anti-Fyb , anti-Lea , anti-Leb , and an anti-KN system antibody. The patient's hemoglobin nadired at 4.4 g/dL. The patient was treated with a single dose of tocilizumab, his hemoglobin stabilized, and he was discharged. DISCUSSION We present a case of HHS proximate to recent SARS-CoV-2 infection with multiple allo and autoantibodies identified. Information on the relationship between SARS-CoV-2 infection and HHS is limited, however, it is possible that inflammation related to COVID-19 could predispose to HHS. Tocilizumab is an approved treatment for COVID-19. Additionally, tocilizumab appears to be a promising treatment option for patients with HHS.
Hyperhemolysis in a patient with sickle cell disease and recent SARS-COV-2 infection, with complex auto- and allo-antibody work-up, successfully treated with tocilizumab.