Recurrence of hemolytic uremic syndrome after renal transplantation

Transplant Proc. 2007 Oct;39(8):2583-5. doi: 10.1016/j.transproceed.2007.08.021.


Non-Shiga toxin-associated hemolytic uremic syndrome (non-Stx-HUS) is a rare disease. The clinical outcome is often unfavorable: 50% of patients progress to end-stage renal failure. Several mutations in complement regulatory genes predispose to non-Stx-HUS. Transplantation outcomes are poor among patients with either mutation in the genes encoding complement H or I factors, with 80% graft loss due to HUS recurrence. In contrast, patients with mutation in the gene encoding MCP have no disease relapse after transplantation. There are no treatment guidelines for non-Stx-HUS recurrence. Herein we have presented 8 patients with non-Stx-HUS recurrence after transplantation during the last 10 years in the South of France. HUS recurrence, which occurred early after transplantation in all but 1 patient, was treated by plasma exchange (PE) with substitution by fresh frozen plasma (FFP). Three patients still treated with long-term plasma therapy have no recurrence at 15, 19, or 24 months. An international registry would help to define new guidelines.

Publication types

  • Multicenter Study

MeSH terms

  • Female
  • Hemolytic-Uremic Syndrome / genetics
  • Hemolytic-Uremic Syndrome / surgery*
  • Humans
  • Kidney Failure, Chronic / surgery*
  • Kidney Transplantation / adverse effects*
  • Male
  • Mutation
  • Recurrence
  • Reoperation
  • Retrospective Studies