Effects of Immunosuppressive Therapy in a Patient with Aplastic Anemia-Paroxysmal Nocturnal Hemoglobinuria (AA-PNH) Syndrome during Ongoing Eculizumab Treatment

摘要

A 65-year-old woman experienced a hemolytic attack triggered by sepsis. She presented with markedly increased CD55-CD59-erythrocytes and the signs of bone marrow failure, which led to a diagnosis of aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome. There was a possibility of increasing hemolysis, as large PNH clones remained after immunosuppressive therapy (IST). Accordingly, eculizumab was first used to control the hemolytic attack followed by IST with antithymocyte globulin and cyclosporine A. The patient was successfully weaned from blood transfusions and has been followed up without any recurrence of hemolytic attacks.