Article
Patients with paroxysmal nocturnal hemoglobinuria demonstrate a prothrombotic clotting phenotype which is improved by complement inhibition with eculizumab.
American journal of hematology - 1 Aug 2020
Macrae Fraser L, Peacock-Young Barnaby, Bowman Polly, Baker Stephen R, Quested Sam, Linton Emma, Hillmen Peter, Griffin Morag, Munir Talha, Payne Daniel, McKinley Claire, Clarke Deborah, Newton Darren J, Hill Anita, Ariëns Robert A S
Abstract excerpt
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hematological disorder, characterized by complement-mediated intravascular hemolysis and thrombosis. The increased incidence of PNH-driven thrombosis is still poorly understood, but unlike other thrombotic disorders, is thought to largely occur through complement-mediated mechanisms. Treatment with a C5 inhibitor, eculizumab, has been shown to significantly...
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