Eculizumab in acute recurrence of thrombotic microangiopathy after renal transplantation.

Abstract

Renal thrombotic microangiopathy (TMA) is a severe complication of systemic lupus erythematosus (SLE), which is associated with the presence of antiphospholipid (aPL) antibodies. In its most fulminant form, TMA leads to a rapid and irreversible end-stage renal failure. Eculizumab, an anti-C5 monoclonal antibody, is a novel therapy of choice for patients with paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome. Here, we report the case of a 27-year-old woman, known for SLE and end-stage renal disease due to fulminant TMA. Both aPL antibodies and antinucleosome antibodies were positive. The patient underwent a living-related kidney transplantation with immediate production of urine. Although serum creatinine was remaining high, a graft biopsy, performed on day 6, demonstrated a TMA recurrence. Despite a treatment with plasma exchange, the situation got worse and dialysis was started. Eculizumab treatment was subsequently administered and renal function improved rapidly. Three months after transplantation, serum creatinine was at 100 μmol/L, without proteinuria. This case illustrates the benefit of eculizumab therapy in a fulminant recurrence of TMA after kidney transplantation, resistant to classical therapy.

• Authors: K Hadaya, S Ferrari-Lacraz, D Fumeaux, F Boehlen, C Toso, S Moll, P-Y Martin, J Villard
• Journal: American journal of transplantation : official journal of the American Society of Transplantation and the American Society of Transplant Surgeons (Am J Transplant) Vol. 11 Issue 11 Pg. 2523-7 (Nov 2011) ISSN: 1600-6143 [Electronic] United States
• PMID: 21831149 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: ©2011 The Authors Journal compilation © 2011 The American Society of Transplantation and the American Society of Transplant Surgeons.

Chemical References

• Antibodies, Antiphospholipid
• Antibodies, Monoclonal, Humanized
• eculizumab

Topics

• Antibodies, Antiphospholipid (blood)
• Antibodies, Monoclonal, Humanized (therapeutic use)
• Female
• Humans
• Kidney Failure, Chronic (etiology)
• Kidney Transplantation (adverse effects)
• Lupus Erythematosus, Systemic (complications)
• Recurrence
• Thrombotic Microangiopathies (drug therapy)