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Long-term effect of the complement inhibitor eculizumab on kidney function in patients with paroxysmal nocturnal
Peter Hillmen1, Modupe Elebute, Richard Kelly
1Department of Hematology, St. James's Institute of Oncology, Leeds, United Kingdom. peter.hillmen@nhs.net
Abstract:
Paroxysmal nocturnal hemoglobinuria (PNH) is a debilitating and life-threatening disease in which lysis of PNH red blood cells frequently manifests with chronic hemolysis, anemia, and thrombosis. Renal damage in PNH is associated with chronic hemosiderosis and/or microvascular thrombosis. We determined the incidence of renal dysfunction or damage, defined by stages of chronic kidney disease (CKD), in a large cohort of PNH patients and evaluated the safety and efficacy of the complement inhibitor eculizumab in altering its progression. Renal dysfunction or damage was observed in 65% of the study population at baseline with 21% of patients with later stage CKD or kidney failure (glomerular filtration rate [GFR]
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