title: [Clinical progress of paroxysmal nocturnal hemoglobinuria].
abstract: Through the applications of high-sensitivity flow cytometry of FLAER and the treatment of eculizumab, it is necessary to understand of paroxysmal nocturnal hemoglobinuria (PNH) from a new point of view. The results of studies demonstrate that treatment with eculizumab alters the natural history of PNH by virtually eradicating thromboembolic complications, inhibiting of intravascular hemolysis and reducing or eliminating transfusion requirements. Eculizumab treatment may also reduce disease-related mortality. This review focuses on the studies to define the relationship between PNH and bone marrow failure syndromes and to characterize the long-term outcome of patients with PNH treated with eculizumab. New therapeutic strategies aimed at controlling extravascular and intravascular hemolysis are discussed.
authors.affiliation: Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China.
authors.forename: Ya-Wen
authors.initials: YW
authors.lastname: Gong
authors.affiliation: Key Laboratory of Thrombosis and Hemostasis of Ministry of Health, Jiangsu Institute of Hematology, The First Affiliated Hospital of Soochow University, Suzhou 215006, Jiangsu Province, China. E-mail: heguangsheng@medmail.com.cn.
authors.forename: Guang-Sheng
authors.initials: GS
authors.lastname: He
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D003182:Complement C5 / Q000276:immunology; D006457:Hemoglobinuria, Paroxysmal / Q000175:diagnosis / Q000628:therapy*; D006801:Humans; D011379:Prognosis
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D003182:Complement C5; C481642:eculizumab