Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab
10.3904/kjm.2020.95.2.124
- Author:
Jung Hyun KIM
1
;
Won Kyung HAN
;
Yu Bum CHOI
;
Hyung Jong KIM
;
Jisu OH
;
Doyeun OH
;
So Young CHONG
Author Information
1. Department of Internal Medicine, CHA Bundang Medical Center, CHA University School of Medicine, Seongnam, Korea
- Publication Type:3
- From:Korean Journal of Medicine
2020;95(2):124-128
- CountryRepublic of Korea
-
Abstract:
Atypical hemolytic uremic syndrome (aHUS) is a rare syndrome characterized by microangiopathic hemolytic anemia, thrombocytopenia, and acute renal injury, which results from uncontrolled complement activation. Delayed diagnosis and treatment of aHUS may result in end-stage renal disease (ESRD) and an associated dependence on dialysis. In extreme cases, it may cause death due to multi-organ failure. Eculizumab, a humanized monoclonal antibody against C5, inhibits the formation of the terminal membrane attack complex and is used to treat aHUS. Here, we report a 46-year-old male patient who suffered from aHUS relapse, despite prior treatment with repeated plasma exchange and hemodialysis. Eculizumab therapy improved his hematologic findings without use of hemodialysis.
