Cold agglutinin disease secondary to severe SARS-CoV-2 treated with eculizumab

Impaired immune response with uncontrolled inflammation and various immunological disorders have been reported during SARS-CoV-2 infection. Here, we report a case of cold agglutinin disease occurring during a severe coronavirus disease 2019 (COVID-19) in a French intensive care unit. A patient was presented with acute respiratory distress syndrome, acute renal failure and haemolytic anaemia. Direct antiglobulin test was positive with a cold agglutinin titre of 1/512. No other cause than COVID-19 explained the occurrence of cold agglutinin disease; however, causality could not be formally established. Persistent anaemia despite transfusion therapy and the short-term life-threatening, prompted the infusion of a monoclonal anti-C5 antibody (eculizumab). Eculizumab therapy quasi-fully resolved haemolysis within a few days, but ultimately the patient died from his severe COVID-19 infection. Data regarding the specific treatment of cold agglutinin disease during COVID-19 are rare. Although additional studies are warranted, eculizumab may be considered in critical situations.

Automated summary

This report presents a case of cold agglutinin disease occurring during severe COVID-19 in a French intensive care unit. The patient presented with acute respiratory distress syndrome, acute renal failure, and hemolytic anemia. Despite transfusion therapy, the patient had persistent anemia and received eculizumab treatment, which partially resolved the hemolysis within a few days. However, the patient ultimately died from the severe COVID-19 infection.