Perioperative Management of Atypical Hemolytic Uremic Syndrome in a Patient on Maintenance Eculizumab Therapy: A Case Report and Review of the Literature.

Abstract

Introduction: Atypical hemolytic uremic syndrome (aHUS) is a rare, complement-mediated disorder characterized by hemolytic anemia, thrombocytopenia, and acute kidney injury. Eculizumab, a monoclonal antibody that inhibits complement component C5, is a cornerstone therapy for aHUS but increases the risk of infections, particularly from encapsulated organisms. Surgical procedures can also raise infection risks or exacerbate thrombotic microangiopathy (TMA). However, data on the perioperative management of patients with aHUS, particularly those receiving eculizumab, remain limited.

Case presentation: A 73-year-old male with a history of prostate cancer presented with acute kidney injury, thrombocytopenia, and hemolysis, leading to a diagnosis of atypical hemolytic uremic syndrome (aHUS). He was treated with eculizumab, which improved kidney function and eliminated the need for dialysis. Four months later, he developed abdominal pain and was found to have gallstones. The patient subsequently underwent a laparoscopic cholecystectomy while continuing maintenance eculizumab therapy. The procedure was performed without any complications, and the patient was discharged in stable condition.

Conclusion: This case report details the successful perioperative management of a patient with aHUS on maintenance eculizumab undergoing cholecystectomy, highlighting the importance of careful management, including continued complement inhibition and infection prevention strategies. Our report emphasizes the need for individualized perioperative care to minimize risks in aHUS patients requiring surgery.