Document Detail

Tailored eculizumab therapy in the management of complement factor H-mediated atypical hemolytic uremic syndrome in an adult kidney transplant recipient: a case report.
MedLine Citation:
PMID:  23195022     Owner:  NLM     Status:  MEDLINE    
Atypical hemolytic uremic syndrome (aHUS) is characterized by thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury (AKI) which frequently progresses to end-stage renal disease (ESRD). In 50% of affected patients, mutations in complement regulatory proteins cause inappropriate complement activation with endothelial injury. Complement factor H (CFH) mutations cause 25% of aHUS cases; these patients have an 80% recurrence risk after kidney transplantation. Eculizumab, an anti-C5 antibody, is effective in limiting hemolysis episodes in patients with aHUS, but less is known about preventing recurrence after kidney transplantation. Herein we report the use of prophylactic eculizumab in an adult with aHUS who underwent kidney transplantation. A 31-year-old female presented with aHUS and progressive AKI associated with low complement 3 level leading to ESRD despite plasmapheresis and corticosteroids. She had a heterozygous nonsense mutation in CFH and reduced plasma CFH levels. She was given preoperative plasmapheresis and eculizumab and underwent living unrelated renal transplantation. Postoperatively, eculizumab was dosed to achieve low functional complement 5 levels and low soluble membrane attack complex levels and she has maintained excellent graft function without aHUS recurrence. We propose that eculizumab with titrated dosing should be used in CFH-mediated aHUS patients who are at a high risk of recurrence.
L Xie; C M Nester; A I Reed; Y Zhang; R J Smith; C P Thomas
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Transplantation proceedings     Volume:  44     ISSN:  1873-2623     ISO Abbreviation:  Transplant. Proc.     Publication Date:  2012 Dec 
Date Detail:
Created Date:  2012-11-30     Completed Date:  2013-05-13     Revised Date:  2013-11-25    
Medline Journal Info:
Nlm Unique ID:  0243532     Medline TA:  Transplant Proc     Country:  United States    
Other Details:
Languages:  eng     Pagination:  3037-40     Citation Subset:  IM    
Copyright Information:
Published by Elsevier Inc.
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MeSH Terms
Acute Kidney Injury / immunology,  surgery*
Antibodies, Monoclonal, Humanized / administration & dosage*
Complement Factor H / antagonists & inhibitors,  genetics,  immunology
Disease Progression
Drug Administration Schedule
Drug Dosage Calculations
Drug Monitoring
Drug Therapy, Combination
Graft Survival / drug effects
Hemolytic-Uremic Syndrome / genetics,  immunology,  prevention & control*
Immunosuppressive Agents / administration & dosage*
Kidney Failure, Chronic / immunology,  surgery*
Kidney Transplantation / immunology*
Living Donors
Recurrence / prevention & control
Time Factors
Treatment Outcome
Reg. No./Substance:
0/Antibodies, Monoclonal, Humanized; 0/Immunosuppressive Agents; 0/complement factor H, human; 80295-65-4/Complement Factor H; A3ULP0F556/eculizumab

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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