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Hematol Rep. 2017 Jun 1;9(2):7053. doi: 10.4081/hr.2017.7053. eCollection 2017 Jun 1.

Atypical Hemolytic Uremic Syndrome: A Brief Review.

Author information

1
Department of Pathology and Laboratory Medicine, University of California, Orange, CA, USA.
2
Department of Internal Medicine, Division of Nephrology and Hypertension, Irvine School of Medicine, University of California, Orange, CA, USA.

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

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