Clinical progress of paroxysmal nocturnal hemoglobinuria.
10.7534/j.issn.1009-2137.2013.06.049
- Author:
Ya-Wen GONG
1
;
Guang-Sheng HE
2
Author Information
1. 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.
2. 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.
- Publication Type:Journal Article
- MeSH:
Antibodies, Monoclonal, Humanized;
therapeutic use;
Complement C5;
immunology;
Hemoglobinuria, Paroxysmal;
diagnosis;
therapy;
Humans;
Prognosis
- From:
Journal of Experimental Hematology
2013;21(6):1627-1630
- CountryChina
- Language:Chinese
-
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.
