Perisurgical induction of eculizumab in a patient with paroxysmal nocturnal hemoglobinuria: Its inhibition of surgery-triggered hemolysis and the consequence of subsequent discontinuation

摘要

Paroxysmal nocturnal hemoglobinuria (PNH) is characterized by complement (C′)-induced lysis of PNH red blood cells (RBCs), which are deficient in the expression of CD55 and CD59. Surgery is one of the major clinical situations that trigger hemolytic attack and thrombosis in PNH. We describe here a case of 64-year-old man with classic PNH complicated by early-stage gastric cancer requiring distal gastrectomy under general anesthesia. We administered humanized monoclonal anti-C5 antibody (eculizumab; Soliris) for a limited period (600mg, once a week×four times) perisurgically. Eculizumab effectively inhibited the C′ system and the patient underwent a curative distal gastrectomy without significant surgery-triggered hemolytic attack. Although discontinuation of eculizumab induced mild hemolysis 2 weeks after the last administration, it was treated conservatively without thrombotic complication. Limited-term induction of eculizumab could be an option for PNH patients with transient and anticipated high risks, with careful preparation for the discontinuation-related risks afterwards.