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Case Reports in Hematology
Volume 2015, Article ID 327980, 3 pages
http://dx.doi.org/10.1155/2015/327980
Case Report

Acute Systolic Heart Failure Associated with Complement-Mediated Hemolytic Uremic Syndrome

1Department of Internal Medicine, The Ohio State University, 395 W. 12th Avenue, Third Floor, Columbus, OH 43210, USA
2Division of Hematology, The Ohio State University, 320 W. 10th Avenue, Columbus, OH 43210, USA

Received 3 September 2015; Accepted 11 October 2015

Academic Editor: Gergely Feher

Copyright © 2015 John L. Vaughn 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

Complement-mediated hemolytic uremic syndrome (otherwise known as atypical HUS) is a rare disorder of uncontrolled complement activation that may be associated with heart failure. We report the case of a 49-year-old female with no history of heart disease who presented with microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. Given her normal ADAMSTS13 activity, evidence of increased complement activation, and renal biopsy showing evidence of thrombotic microangiopathy, she was diagnosed with complement-mediated HUS. She subsequently developed acute hypoxemic respiratory failure secondary to pulmonary edema requiring intubation and mechanical ventilation. A transthoracic echocardiogram showed evidence of a Takotsubo cardiomyopathy with an estimated left ventricular ejection fraction of 20%, though ischemic cardiomyopathy could not be ruled out. Treatment was initiated with eculizumab. After several failed attempts at extubation, she eventually underwent tracheotomy. She also required hemodialysis to improve her uremia and hypervolemia. After seven weeks of hospitalization and five doses of eculizumab, her renal function and respiratory status improved, and she was discharged in stable condition on room air and independent of hemodialysis. Our case illustrates a rare association between acute systolic heart failure and complement-mediated HUS and highlights the potential of eculizumab in stabilizing even the most critically-ill patients with complement-mediated disease.