Atypical hemolytic-uremic syndrome: clinical presentation, diagnosis and treatment

Korotchaeva Y.V.: Sechenov University, Moscow

Kozlovskaya N.L.: Eramishancev City Clinical Hospital, Moscow

Demyanova K.: Eramishantsev City Clinical Hospital

Alexeeva M.: Eramishantsev City Clinical Hospital

Skvortsov A.: Moscow State University, Moscow

Moiseev S.: Tareev Clinic of Internal Diseases, Sechenov First Moscow State Medical University, Moscow, Russia

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Summary

Atypical hemolytic-uremic syndrome (aHUS) is a rare disease caused by uncontrolled activation of the alternative complement pathway and characterized by acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia. Up to one third of aHUS patients present with extrarenal manifestations including central nervous system, lung, liver, and gastrointestinal tract involvement. Identified triggers of aHUS include various complement activating disorfers, e.g. complicated pregnancy. Eculizumab, the terminal complement C5 inhibitor, is targeted treatment approved for patients with aHUS. The authors present two cases of aHUS and review its clinical presentation, diagnosis and treatment.

Key words

Thrombotic microangiopathy, atypical hemolytic-uremic syndrome, eculizumab.