Recurrence of HUS due to CD46/MCP mutation after renal transplantation: a role for endothelial microchimerism

Am J Transplant. 2007 Aug;7(8):2047-51. doi: 10.1111/j.1600-6143.2007.01888.x.

Abstract

Mutations in the gene of the membrane cofactor protein (MCP/CD46), a complement regulatory protein, were recently described as a cause of hemolytic uremic syndrome (HUS). MCP is a transmembrane glycoprotein expressed in kidneys; therefore, the transplantation of a normal kidney should not be complicated by HUS recurrence. However, we report the case of a 32-year-old woman with an MCP mutation who developed a recurrence of HUS after renal transplantation. We found that she had vascular microchimerism of endothelial cells. We suggest that recurrence may be favored by vascular microchimerism, in which the mutated protein is produced in the in the kidney graft by endothelial cells originating from recipient.

Publication types

  • Case Reports

MeSH terms

  • Acute Kidney Injury / surgery
  • Adult
  • Biopsy
  • Chimerism*
  • DNA / genetics*
  • Endothelial Cells / pathology*
  • Enzyme-Linked Immunosorbent Assay
  • Female
  • Follow-Up Studies
  • Hemolytic-Uremic Syndrome / genetics*
  • Hemolytic-Uremic Syndrome / metabolism
  • Hemolytic-Uremic Syndrome / pathology
  • Humans
  • Kidney Transplantation*
  • Membrane Cofactor Protein / genetics*
  • Membrane Cofactor Protein / immunology
  • Membrane Cofactor Protein / metabolism
  • Mutation*
  • Postoperative Complications
  • Recurrence
  • Reverse Transcriptase Polymerase Chain Reaction

Substances

  • CD46 protein, human
  • Membrane Cofactor Protein
  • DNA