Critical appraisal of eculizumab for atypical hemolytic uremic syndrome

Lilian M.Pereira Palma, Craig B. Langman*

*Corresponding author for this work

Research output: Contribution to journalReview article

18 Scopus citations

Abstract

The biology of atypical hemolytic uremic syndrome has been shown to involve inability to limit activation of the alternative complement pathway, with subsequent damage to systemic endothelial beds and the vasculature, resulting in the prototypic findings of a thrombotic microangiopathy. Central to this process is the formation of the terminal membrane attack complex C5b-9. Recently, application of a monoclonal antibody that specifically binds to C5, eculizumab, became available to treat patients with atypical hemolytic uremic syndrome, replacing plasma exchange or infusion as primary therapy. This review focuses on the evidence, based on published clinical trials, case series, and case reports, on the efficacy and safety of this approach.

Original languageEnglish (US)
Pages (from-to)39-72
Number of pages34
JournalJournal of Blood Medicine
Volume7
DOIs
StatePublished - Apr 12 2016

Keywords

  • Acute kidney injury
  • Alternative complement pathway
  • Complement blockade
  • ESRD
  • Kidney
  • Thrombotic microangiopathy

ASJC Scopus subject areas

  • Hematology

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