Innovative therapeutic approach: sequential treatment with plasma exchange and eculizumab in a pregnant woman affected by atypical hemolytic-uremic syndrome

Transfus Apher Sci. 2014 Oct;51(2):134-6. doi: 10.1016/j.transci.2014.08.027. Epub 2014 Sep 16.

Abstract

The atypical HUS (aHUS) is a rare genetic disease, with poor prognosis, characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. This syndrome is often related to mutations in the genes encoding complement regulatory proteins. A 26-year-old woman with homozygous mutation in complement factor H (CFH) developed a relapse of aHUS at 17th week of pregnancy. Despite treatment with plasma exchange (PEX), at the 26th week of gestation eculizumab was started. The sequential treatment with eculizumab after PEX was well tolerated and it has led to clinical remission.

Keywords: Atypical HUS; Eculizumab; Transfusion practices.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Humanized / administration & dosage*
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Atypical Hemolytic Uremic Syndrome / therapy*
  • Complement Factor H / genetics
  • Female
  • Genetic Diseases, Inborn / genetics
  • Genetic Diseases, Inborn / therapy*
  • Homozygote
  • Humans
  • Mutation
  • Plasma Exchange*
  • Pregnancy
  • Pregnancy Complications, Hematologic / genetics
  • Pregnancy Complications, Hematologic / therapy*

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement Factor H
  • eculizumab