S-473 Treatment of a patient of atypical hemolytic uremic syndrome with plasma pharesis = S-473 Treatment of a patient of atypical hemolytic uremic syndrome with plasma pharesis

다국어 초록 (Multilingual Abstract)

Hemolytic uremic syndrome (HUS) is defined as the triad of microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. Approximately 90 percent of cases of HUS are due to Shiga toxin-producing E. coli (STEC), Streptococcus pneumonia, and Shigella dysenteriae type 1 infection. But one of the other rare causes of HUS is genetic mutations of complement factors which induced Complement-mediated HUS, atypical HUS. Herein, we report an adult case of atypical HUS who was successfully treated with plasmapheresis. A 37-year-old man was admitted to the hospital because of fever, abdominal pain, diarrhea and whole body skin rash. He didn’t have any history of drug-intake or underlying disease. He was first treated with antibiotics and vasopressors as septic shock due to colitis. Because acute kidney injury, thrombocytopenia, microangiopathic hemolytic anemia and other organ failures were progressively developed during the treatment, we diagnosed him as HUS. While the evaluation of stool for pathogens was in progress, We applied continuous renal replacement therapy (CRRT) and plasmapheresis. After excluding other auto-immune disorders and infection of virus and bacteria, We could clinically diagnosed him as atypical HUS. After applying CRRT and plasmapheresis, Hematologic complication and renal failure were improved without applying other agents like Eculizumab. Atypical HUS, usually triggered by infection, is developed by causing dysregulation of alternative complement pathway which makes the membrane attack complex and leads to hematologic complications as hemolytic anemia and coagulopathy. Treatment of atypical HUS is composed of supportive therapy, plasma infusion or exchange, and Eculizumab, a humanized monoclonal antibody that inhibits terminal complement activation. And, plasma exchange therapy, or plasmapheresis can be used empirically in purpose of removal of procoagulant like mutant complements and restoration of complement function. In this case, we used a plasmapheresis and led to good clinical outcomes in atypical HUS.