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KMID : 0882420200950020124
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Korean Journal of Medicine
2020 Volume.95 No. 2 p.124 ~ p.128
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Atypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab
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Kim Jung-Hyun
Han Won-Kyung
Choi Yu-Bum
Kim Hyung-Jong
Oh Ji-Su
Oh Do-Yeun
Chong So-Young
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Abstract
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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.
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KEYWORD
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Atypical hemolytic uremic syndrome, Eculizumab
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