title: Effects of immunosuppressive therapy in a patient with aplastic anemia-paroxysmal nocturnal hemoglobinuria (AA-PNH) syndrome during ongoing eculizumab treatment.
abstract: 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.
authors.affiliation: Department of Hematology and Clinical Immunology, Nishi-Kobe Medical Center, Japan.
authors.forename: Jin
authors.initials: J
authors.lastname: Asano
authors.forename: Ryosuke
authors.initials: R
authors.lastname: Ueda
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Tanaka
authors.forename: Isaku
authors.initials: I
authors.lastname: Shinzato
authors.forename: Toshiro
authors.initials: T
authors.lastname: Takafuta
mesh_terms: D000368:Aged; D000741:Anemia, Aplastic / Q000188:drug therapy* / Q000209:etiology / Q000628:therapy; D000900:Anti-Bacterial Agents / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D000961:Antilymphocyte Serum / Q000627:therapeutic use; D016470:Bacteremia / Q000150:complications / Q000188:drug therapy; D001803:Blood Transfusion; D003131:Combined Modality Therapy; D051056:Complement Inactivating Agents / Q000627:therapeutic use*; D016572:Cyclosporine / Q000627:therapeutic use; D004927:Escherichia coli Infections / Q000150:complications / Q000188:drug therapy; D005260:Female; D016179:Granulocyte Colony-Stimulating Factor / Q000627:therapeutic use; D006457:Hemoglobinuria, Paroxysmal / Q000150:complications / Q000188:drug therapy*; D006461:Hemolysis; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D000078224:Lenograstim; D011994:Recombinant Proteins / Q000627:therapeutic use; D012772:Shock, Septic / Q000209:etiology; D013601:T-Lymphocytes; D014552:Urinary Tract Infections / Q000150:complications / Q000188:drug therapy
chemical_list: D000900:Anti-Bacterial Agents; D061067:Antibodies, Monoclonal, Humanized; D000961:Antilymphocyte Serum; D051056:Complement Inactivating Agents; D007166:Immunosuppressive Agents; D011994:Recombinant Proteins; D016179:Granulocyte Colony-Stimulating Factor; D000078224:Lenograstim; D016572:Cyclosporine; C481642:eculizumab