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An adult case of atypical hemolytic uremic syndrome presented with posterior reversible encephalopathy syndrome: Successful response to late-onset eculizumab treatment

Serife Solmaz Medeni, Sinem Namdaroglu, Tugba Cetintepe, Can Ozlu, Funda Tasli, Zehra Hilal Adibelli, Oktay Bilgir, Erhan Tatar
  • Serife Solmaz Medeni
    Department of Hematology, Bozyaka Teaching and Research Hospital, Izmir, Turkey | solmazserife@yahoo.com
  • Sinem Namdaroglu
    Department of Hematology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Tugba Cetintepe
    Department of Hematology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Can Ozlu
    Department of Hematology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Funda Tasli
    Department of Pathology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Zehra Hilal Adibelli
    Department of Radiology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Oktay Bilgir
    Department of Hematology, Bozyaka Teaching and Research Hospital, Izmir, Turkey
  • Erhan Tatar
    Department of Nephrology, Bozyaka Teaching and Research Hospital, Izmir, Turkey

Abstract

Atypical hemolytic uremic syndrome is a rare and progressive disease caused by uncontrolled alternative complement activation. Dysregulatıon of the complement activation results in thrombotic microangiopathy and multiorgan damage. A 29-yearold woman who was admitted with complaints of vomiting and headache was detected to have acute renal failure with microangiopathic hemolytic anemia (MAHA). After the diagnosis of atypical hemolytic uremic syndrome (aHUS), she was treated with plasma exchange (PE) and hemodialysis (HD). She has experienced hypertensionrelated posterior reversible encephalopathy syndrome (PRES) at the second plasma exchange. She was initiated on eculizumab therapy because of no response to PE on the 34th days. Her renal functions progressively improved with eculizumab treatment. Dependence on dialysis was over by the 4th month. Dialysis free-serum Creatinine level was 2.2 mg/dL [glomerular filtration rate (e-GFR): 30 mL/min/1.73 m2] after 24 months. Neurological involvement (PRES, etc.) is the most common extrarenal complication and a major cause of mortality and morbidity from aHUS. More importantly, we showed that renal recovery may be obtained following late-onset eculizumab treatment in patient with aHUS after a long dependence on hemodialysis.

Keywords

Atypical hemolytic uremic syndrome, eculizumab, posterior reversible encephalopathy syndrome, hemodialysis.

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Submitted: 2017-12-26 23:22:22
Published: 2018-09-24 14:15:31
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Copyright (c) 2018 Serife Solmaz Medeni, Sinem Namdaroglu, Tugba Cetintepe, Can Ozlu, Funda Taslı, zehra Adıbelli, Oktay Bilgir, Erhan Tatar

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