Thrombotic microangiopathy (TMA) is characterized by thrombocytopenia, microangiopathic hemolytic anemia and end organ damage from microvascular thrombosis. Atypical hemolytic uremic syndrome (aHUS) is one form of TMA that occurs because of pathologic complement activation. Mutations in the genes coding for complement components predispose to hyperactivation of the alternative complement pathway. Therapeutic complement inhibition is effective in aHUS. Eculizumab, a monoclonal humanized anti-C5 antibody, has shown success in patients with aHUS.