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Hematology Reports
Volume 9
Issue 2
10.4081/hr.2017.7053
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Review

Atypical Hemolytic Uremic Syndrome: A Brief Review

by
Kuixing Zhang
1,*,
Yuxin Lu
1,
Kevin T. Harley
2 and
Minh-Ha Tran
1
1
Department of Pathology and Laboratory Medicine, University of California Irvine Medical Center, 101 The City Drive South, Bldg 1, 3rd Flr, Rm 3003, Orange, CA 92868, USA
2
Department of Internal Medicine, Division of Nephrology and Hypertension, Irvine School of Medicine, University of California, Orange, CA 92868, USA
*
Author to whom correspondence should be addressed.
Hematol. Rep. 2017, 9(2), 7053; https://doi.org/10.4081/hr.2017.7053
Submission received: 17 January 2017 / Revised: 7 February 2017 / Accepted: 8 February 2017 / Published: 1 June 2017

Abstract

Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. The histopathologic lesions of aHUS include thrombotic microangiopathy involving the glomerular capillaries and thrombosis involving arterioles or interlobar arteries. Extra-renal manifestations occur in up to 20% of patients. The majority of aHUS is caused by complement system defects impairing ordinary regulatory mechanisms. Activating events therefore lead to unbridled, ongoing complement activity producing widespread endothelial injury. Pathologic mutations include those resulting in loss-of-function in a complement regulatory gene (CFH, CFI, CD46 or THBD) or gain-of-function in an effector gene (CFB or C3). Treatment with the late complement inhibitor, eculizumab—a monoclonal antibody directed against C5—is effective.
Keywords: atypical hemolytic uremic syndrome; genetics atypical hemolytic uremic syndrome; genetics

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MDPI and ACS Style

Zhang, K.; Lu, Y.; Harley, K.T.; Tran, M.-H. Atypical Hemolytic Uremic Syndrome: A Brief Review. Hematol. Rep. 2017, 9, 7053. https://doi.org/10.4081/hr.2017.7053

AMA Style

Zhang K, Lu Y, Harley KT, Tran M-H. Atypical Hemolytic Uremic Syndrome: A Brief Review. Hematology Reports. 2017; 9(2):7053. https://doi.org/10.4081/hr.2017.7053

Chicago/Turabian Style

Zhang, Kuixing, Yuxin Lu, Kevin T. Harley, and Minh-Ha Tran. 2017. "Atypical Hemolytic Uremic Syndrome: A Brief Review" Hematology Reports 9, no. 2: 7053. https://doi.org/10.4081/hr.2017.7053

APA Style

Zhang, K., Lu, Y., Harley, K. T., & Tran, M. -H. (2017). Atypical Hemolytic Uremic Syndrome: A Brief Review. Hematology Reports, 9(2), 7053. https://doi.org/10.4081/hr.2017.7053

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