Open AccessAtypical Hemolytic Uremic Syndrome Successfully Treated with Eculizumab
Author(s) -
Jung Hyun Kim,
Won Kyung Han,
Yeo Jin Choi,
Hyung Jong Kim,
Jun-Hyok Oh,
Doyeun Oh,
So Young Chong
Publication year - 2020
Publication title -
the korean journal of medicine
Language(s) - English
Resource type - Journals
eISSN - 2289-0769
pISSN - 1738-9364
DOI - 10.3904/kjm.2020.95.2.124
Subject(s) - eculizumab , atypical hemolytic uremic syndrome , microangiopathic hemolytic anemia , medicine , thrombotic microangiopathy , hemodialysis , dialysis , hemolytic anemia , complement system , end stage renal disease , monoclonal , acute kidney injury , anemia , gastroenterology , disease , monoclonal antibody , immunology , antibody , thrombotic thrombocytopenic purpura , platelet
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.