[Successful eculizumab treatment for multiple coronary thrombosis complicated in paroxysmal nocturnal hemoglobinuria]

Ayuko Taniguchi1, Takayuki Ikezoe, Asako Takeuchi

  • 1Department of Hematology and Respiratory Medicine, Kochi Medical School, Kochi University.

We herein report a rare case of paroxysmal nocturnal hemoglobinuria (PNH) who repeatedly developed coronary arterial thromboembolism. Anticoagulant therapies including heparin, aspirin as an antiplatelet agent and even drug-eluting stent placement in the coronary artery failed to prevent the recurrence of ischemic heart disease. Of note, initiating the administration of a humanized anti-C5 antibody, eculizumab, achieved prompt thrombolysis and maintenance treatment with eculizumab prevented the recurrence of thromboembolic disease in this patient. Taking these observations together, we suggest that the use of eculizumab be considered for treatment or prevention of arterial thrombosis complicated by PNH, although arterial thrombosis is an extremely rare event in the Japanese population.

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