Pre-emptive eculizumab and plasmapheresis for renal transplant in atypical hemolytic uremic syndrome

Clin J Am Soc Nephrol. 2011 Jun;6(6):1488-94. doi: 10.2215/CJN.10181110. Epub 2011 May 26.

Abstract

The case of a 12-year-old with a hybrid CFH/CFHL1 gene and atypical hemolytic uremic syndrome (aHUS) that had previously developed native kidney and then renal allograft loss is reported. This case illustrates the relatively common occurrence of renal loss from the late presentation of aHUS. Also presented is a protocol for the pre-emptive use of eculizumab and plasmapheresis as part of a renal transplant plan for the treatment of aHUS in patients deemed at high risk for recurrent disease. This protocol was a result of a multidisciplinary approach including adult and pediatric nephrology, transplant surgery, transfusion medicine, and infectious disease specialists. This protocol and the justifications and components of it can function as a guideline for the treatment of a group of children that have waited in limbo for the first U.S. transplant to open the door to this type of definitive care for this devastating disease.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Monoclonal / administration & dosage*
  • Antibodies, Monoclonal, Humanized
  • Atypical Hemolytic Uremic Syndrome
  • Child
  • Drug Administration Schedule
  • Female
  • Graft Survival
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / therapy*
  • Humans
  • Kidney Transplantation*
  • Mutant Chimeric Proteins / genetics
  • Patient Care Team
  • Plasmapheresis*
  • Preoperative Care
  • Recurrence
  • Renal Dialysis
  • Reoperation
  • Time Factors
  • Treatment Outcome

Substances

  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • CFH-CFHL1 protein, human
  • Mutant Chimeric Proteins
  • eculizumab