Adult Post-Kidney Transplant Familial Atypical Hemolytic Uremic Syndrome Successfully Treated With Eculizumab: A Case Report and Literature Review

Am J Ther. 2016 Jul-Aug;23(4):e1110-5. doi: 10.1097/MJT.0000000000000133.

Abstract

Hemolytic uremic syndrome is the triad of nonimmune microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. When not associated with enteric infection, it is classified as atypical hemolytic uremic syndrome (aHUS) and carries a worse outcome with high mortality rate and up to 50% of the survivors will end up with end-stage renal disease. Renal transplant was restricted to a very small percentage of patients due to high recurrence rate posttransplant that approaches 90%. Our case describes a posttransplant adult patient with familial aHUS whom was successfully treated with eculizumab. We also reviewed all other reported cases of adult posttransplant aHUS, both familial and sporadic, which were treated with eculizumab. In summary, eculizumab might expand the utility of renal transplant for patients with end-stage renal disease due to aHUS.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal, Humanized / therapeutic use*
  • Atypical Hemolytic Uremic Syndrome / drug therapy*
  • Atypical Hemolytic Uremic Syndrome / etiology*
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Complement System Proteins / genetics
  • Humans
  • Kidney Transplantation / adverse effects*
  • Male
  • Middle Aged
  • Mutation

Substances

  • Antibodies, Monoclonal, Humanized
  • Complement System Proteins
  • eculizumab