Eyal Kristal MD, Shani Arotzker MD, Michael Geylis MD, Siham Elamour MD, Galina Ling MD, Ruth Schreiber MD
Atypical hemolytic uremic syndrome (aHUS) is a rare, life-threatening thrombotic microangiopathy (TMA) characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury. In contrast to typical HUS, which is triggered by Shiga toxin–producing bacteria, aHUS results from genetic or acquired dysregulation of the alternative pathway (AP) of the complement system and has an estimated incidence of 1–2 cases per million per year [1]. Early recognition is critical, as terminal complement inhibition with C5 blockers significantly improves renal and overall outcomes [2,3].