Eculizumab therapy in paroxysmal nocturnal haemoglobinuria patient undergoing aortic valve surgery

Abstract

Paroxysmal nocturnal haemoglobinuria is a rare disorder characterized by haemolytic anaemia and pancytopaenia. The use of cardiopulmonary bypass can lead to a haemolytic crisis in patients with paroxysmal nocturnal haemoglobinuria due to activation of complement-mediated haemolysis. We report the successful management of a 69-year-old man undergoing aortic valve replacement with standard heparin-protamine protocol by using eculizumab, a monoclonal antibody of complement factor C5. The surgery was performed without triggering a haemolytic crisis, and the patient was discharged from the hospital without major complications.