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Updated: Jul 11, 2026

Methods for Quantitative Detection of Antibody-induced Complement Activation on Red Blood Cells
Published on: January 29, 2014
Eculizumab as first-line therapy for atypical hemolytic uremic syndrome
Martin Christmann1, Matthias Hansen2, Carsten Bergmann3
1Klinik für Kinder- und Jugendmedizin, Pädiatrische Intensivstation, Goethe-Universität, Frankfurt/Main, Germany; martin.christmann@kispi.uzh.ch.
Abstract:
Atypical hemolytic uremic syndrome (aHUS) is a genetic, life-threatening, chronic disease that can affect patients of all ages. aHUS is caused by uncontrolled complement activation due to genetic defects of complement regulation. Plasma exchange or infusion has been used to manage aHUS and may transiently maintain hematologic variables in some patients, but as the underlying complement dysregulation persists, end-stage renal disease or death occurs in 33% to 40% of patients during the first clinical manifestation. Here we present a pediatric case showing that first-line eculizumab treatment successfully blocked the progression of thrombotic microangiopathy in aHUS.
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