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Case Reports in Hematology
Volume 2015, Article ID 673195, 4 pages
http://dx.doi.org/10.1155/2015/673195
Case Report

Improvement of Renal Function by Long-Term Sustained Eculizumab Treatment in a Patient with Paroxysmal Nocturnal Hemoglobinuria

1Department of Medical Sciences, Faculty of Medicine, University of Tsukuba, Tsukuba 305-8575, Japan
2Department of Hematology, Faculty of Medicine, University of Tsukuba, Tsukuba 305-8575, Japan
3Department of Internal Medicine, Hitachi, Ltd., Hitachinaka General Hospital, Hitachinaka 312-0057, Japan

Received 4 April 2015; Accepted 27 May 2015

Academic Editor: Ramon Tiu

Copyright © 2015 Haruhiko Ninomiya et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Chronic kidney disease (CKD) is one of the major manifestations of paroxysmal nocturnal hemoglobinuria (PNH). CKD in PNH is induced mainly by intravascular hemolysis of PNH-affected red blood cells (RBC) missing the glycosylphosphatidylinositol-anchored proteins with complement-regulatory activities, CD55 and CD59. CKD develops by heme absorption in the proximal tubules resulting in the interstitial deposition of iron in the kidneys. We administered eculizumab to a patient with PNH, who was one of 29 patients enrolled in the AEGIS clinical trial, an open-label study of eculizumab in Japan. The patient was complicated by stage 3 CKD with impaired estimated glomerular filtration rate (eGFR), at grade G3b, and had obvious proteinuria (2-3+, 1-2 g/day). In a two-year extension to the 12-week AEGIS study, eGFR improved significantly, and the eGFR has since been maintained at grade G2 without proteinuria by sustained eculizumab treatment (>6 years). Renal function improved and maintained by long-term sustained eculizumab treatment, presumably by clearance of iron from the kidney as well as inhibition of the production of anaphylatoxin C5a, even in advanced stages of CKD, is one of the benefits of eculizumab treatment in PNH.