RESEARCH ARTICLE


Continued Eculizumab Therapy for Persistent Atypical Hemolytic Uremic Syndrome



Arif Asif 1, *, Syed S. Haqqie1, Ketan Ghate1, Roy O Mathew1, Tushar Vachharajani2 , Ali Nayer3
1 Division of Nephrology and Hypertension, Albany Medical College and Stratton VA Medical Center, Albany, NY, USA
2 Division of Nephrology and Hypertension W.G. (Bill) Hefner Veterans Affairs Medical Center, Salisbury, NC, USA
3 Division of Nephrology and Hypertension, University of Miami Miller School of Medicine, Miami, FL, USA


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© Mrkoci and Chretien et al.; Licensee

open-access license: This is an open access article licensed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

* Address correspondence to this author at the Division of Nephrology and Hypertension, Albany Medical College, 25 Hackett Blvd; Albany, NY 12208, USA; Tel: 518-269-0769; Fax: 518-269-0769; E-mail: asifa@mail.amc.edu


Abstract

Atypical hemolytic uremic syndrome (atypical HUS) is characterized by endothelial injury and microvascular thrombosis resulting in microangiopathic hemolytic anemia, thrombocytopenia, and ischemic injury to organs, especially the kidney. Activation of complements is involved in the pathogenesis of atypical HUS. Eculizumab, a neutralizing monoclonal antibody directed against complement C5, has shown salutary effects in patients with atypical HUS. In this report, we present a 23-year-old man with atypical HUSwho was treated with eculizumab. During the first four weeks of treatment, eculizumab failed to achieve a remission. Microangiopathic hemolytic anemia and thrombocytopenia persisted, while renal function deteriorated necessitating initiation of hemodialysis. Continuation of eculizumab therapy, however, led to marked improvement in hemolytic anemia, thrombocytopenia, and renal function. After 10 weeks of eculizumab therapy, hemodialysis was discontinued. At 5-month follow-up, serum creatinine was 1.1 mg/dL with continued eculizumab therapy every other week. In addition, platelet count was normal, while there was no evidence of hemolysis. We conclude that in patients with persistent atypical HUS continued treatment with eculizumab can be helpful in achieving remission.

Keywords : Atypical hemolytic uremic syndrome, thrombotic microangiopathy, hemolytic uremic syndrome, eculizumab, complements.