Recurrence of HUS Due to CD46/MCP Mutation After Renal Transplantation: A Role for Endothelial Microchimerism

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 complic...

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Bibliographic Details
Published in:American journal of transplantation 2007-08, Vol.7 (8), p.2047-2051
Main Authors: Frémeaux‐Bacchi, V., Arzouk, N., Ferlicot, S., Charpentier, B., Snanoudj, R., Dürrbach, A.
Format: Article
Language:English
Subjects:
Acute Kidney Injury - surgery
Adult
Biological and medical sciences
Biopsy
CD46/MCP
Chimerism
DNA - genetics
Endothelial Cells - pathology
Enzyme-Linked Immunosorbent Assay
Female
Follow-Up Studies
Hematologic and hematopoietic diseases
hemolytic uremic syndrome
Hemolytic-Uremic Syndrome - genetics
Hemolytic-Uremic Syndrome - metabolism
Hemolytic-Uremic Syndrome - pathology
Humans
Kidney Transplantation
Medical sciences
Membrane Cofactor Protein - genetics
Membrane Cofactor Protein - immunology
Membrane Cofactor Protein - metabolism
microchimerism
Mutation
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
Platelet diseases and coagulopathies
Postoperative Complications
Recurrence
Renal failure
renal transplantation
Reverse Transcriptase Polymerase Chain Reaction
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
Surgery of the urinary system
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Summary: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. This kidney transplanted into a 32‐year‐old woman with mutations in complement regulatory protein CD46/MCP developed recurrent HUS in the kidney, associated with microchimerism of vascular endothelial cells from the recipient in the donor blood vessels.
ISSN:1600-6135
1600-6143
DOI:10.1111/j.1600-6143.2007.01888.x