Abstract
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome of hemolysis, thrombocytopenia, and renal insufficiency often accompanied with multiple organ dysfunction and other complications. Because aHUS shares many of the presenting characteristics of the other thrombotic microangiopathies, and confirmatory diagnostic tests are not yet available, the diagnosis relies heavily on the recognition of a clinical syndrome consistent with the diagnosis in the absence of signs of an alternate cause of thrombotic microangiopathy.
Treatment of aHUS consists of early plasma therapy followed by an eculizumab therapy as soon as the aHUS diagnosis is confirmed. Eculizumab has dramatically improved the prognosis of patients with aHUS and general approach to thrombotic microangiopathy treatment as well.