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Semin Thromb Hemost. 2014 Jun;40(4):444-64. doi: 10.1055/s-0034-1376153. Epub 2014 Jun 9.

Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches.

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Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria.
Department of Nephrology and Immunology, ITUN and INSERM UMR S-1064, CHU de Nantes, Nantes, France.
Service de Nephrologie et de Transplantation Rénale, Hôpital Foch, Suresnes, France.
Division of Nephrology, The Hospital for Sick Children, Toronto, Ontario, Canada.
INSERM U1138, Cordelier Research Center "Complement and Diseases" Paris, France.


Thrombotic microangiopathy (TMA) is a rare but severe disorder characterized by endothelial cell activation and thrombus formation. It manifests with the triad of hemolytic anemia, thrombocytopenia, and organ failure. Prompt diagnosis and treatment initiation are crucial for long-term outcome. TMA often manifests subsequent to infectious events, of which (enterohemorrhagic) Escherichia coli is the most frequently reported. TMA also occurs on the background of genetic/autoimmune defects in the complement system (atypical hemolytic uremic syndrome [aHUS]) and underlying conditions, such as pregnancy, transplantation, drugs, other glomerulopathies, vasculitides, or metabolic defects. Complement activation or defects in its regulation have now been described in an increasing number of acquired diseases with TMA. Coinciding with this expanding spectrum of complement-mediated diseases, the question arises which patients might benefit from a complement-targeted therapy. Success of therapy depends on the individual contribution of complement activation in disease pathogenesis. The advent of eculizumab, a monoclonal antibody that blocks terminal complement activation, has markedly improved outcome and quality of life in patients with aHUS. This review discusses the contribution of complement and highlights its complex interaction with inflammation, coagulation, and the endothelium. Treatment experiences focusing on eculizumab therapy are discussed in detail across the emerging spectrum of complement-mediated thrombotic microangiopathies.

[Indexed for MEDLINE]

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