Prophylactic use of eculizumab during surgery in chronic cold agglutinin disease

• Published in: BMJ case reports Vol. 2017; p. bcr-2016-219066
• Main Authors: Tjønnfjord, Eirik, Vengen, Øystein A, Berentsen, Sigbjørn, Tjønnfjord, Geir Erland
• Format: Journal Article
• Language: English
• Published: England BMJ Publishing Group LTD 09.05.2017; BMJ Case Reports
• Series: Case Report
• Subjects: Aged; Anemia; Anemia, Hemolytic, Autoimmune - blood; Anemia, Hemolytic, Autoimmune - drug therapy; Antibodies, Monoclonal, Humanized - administration & dosage; Antibodies, Monoclonal, Humanized - therapeutic use; Antigens; Aortic Valve; Blood; Bone marrow; Cardiac Valve Annuloplasty; Disease; Female; Humans; Infections; Novel Treatment (New Drug/Intervention; Established Drug/Procedure in New Situation); Preoperative Care; Scandinavia; Seasonal variations; Surgery; White; Drug therapy related to surgery; Haematology (incl blood transfusion); Haematology (drugs and medicines)
• ISSN: 1757-790X; 1757-790X
• DOI: 10.1136/bcr-2016-219066

Primary chronic cold agglutinin disease (CAD) is an autoimmune haemolytic anaemia in which a specific bone marrow lymphoproliferative disorder causes production of cold agglutinins (CA). Binding of CA to erythrocyte surface antigens results in a predominantly extravascular haemolysis that is entirely complement dependent. Because of complement activation, exacerbations are common during febrile infections, trauma or major surgery. Involvement of the terminal complement pathway with C5-mediated intravascular haemolysis is probably not prominent in stable disease but is supposed to be of importance in exacerbations following acute phase reaction.We report on a patient with CAD prone to exacerbation of haemolysis during acute phase reactions who was scheduled for cardiac surgery. To prevent her having an exacerbation of haemolysis, we chose to treat her prophylactically with eculizumab along with the usual perioperative precautions. Aortic valve replacement was undertaken with full cardiopulmonary bypass at normothermia. The procedure was successful; no exacerbation of haemolysis was observed, and transfusion requirements did not exceed what could be expected.