The Importance of Eculizumab in the Treatment of Atypical Hemolytic Uremic Syndrome

Cureus. 2022 Jun 8;14(6):e25743. doi: 10.7759/cureus.25743. eCollection 2022 Jun.

Abstract

In most cases, the atypical hemolytic uremic syndrome (aHUS) is a genetic disease resulting from defects in the regulation of the complement cascade, which conditions the development of thrombotic microangiopathy. Clinically, aHUS presents with acute kidney injury, thrombocytopenia, and microangiopathic hemolytic anemia, with frequent renal and extrarenal thrombotic phenomena. Early diagnosis and treatment are essential. Plasmapheresis is an alternative treatment being frequently used considering the high cost of eculizumab, despite its lower clinical efficacy. This article describes the clinical case of a patient admitted to intensive care with a personal history of aHUS diagnosed 10 years ago, with recurrent aHUS triggered by viral infection. The patient presented with acute kidney injury and thrombocytopenia. Despite the institution of admission of plasmapheresis, the clinical evolution was only favorable after the administration of eculizumab, highlighting the importance of early initiation of this therapy.

Keywords: ahus; eculizumab; mortality; plasmapheresis; prognosis.

Publication types

  • Case Reports