Eculizumab for atypical hemolytic uremic syndrome recurrence in renal transplantation
J Zuber, M Le Quintrec, S Krid, C Bertoye, V Gueutin, A Lahoche, N Heyne, G Ardissino, V Chatelet, L-H Noël, M Hourmant, P Niaudet, V Frémeaux-Bacchi, E Rondeau, C Legendre, C Loirat, French Study Group for Atypical HUS, M Buchler, S Burtey, Y Delmas, G Deschênes, F Fakhouri, A Karras, B Knebelmann, B Moulin, C Pouteil-Noble, F Provost, J Zuber, M Le Quintrec, S Krid, C Bertoye, V Gueutin, A Lahoche, N Heyne, G Ardissino, V Chatelet, L-H Noël, M Hourmant, P Niaudet, V Frémeaux-Bacchi, E Rondeau, C Legendre, C Loirat, French Study Group for Atypical HUS, M Buchler, S Burtey, Y Delmas, G Deschênes, F Fakhouri, A Karras, B Knebelmann, B Moulin, C Pouteil-Noble, F Provost
Abstract
Eculizumab (anti-C5) has been sporadically reported as an efficient therapy for atypical hemolytic uremic syndrome (aHUS). However, the lack of series precludes any firm conclusion about the optimal use of anti-C5 for preventing or treating aHUS posttransplant aHUS recurrence. We thoroughly studied 22 renal transplant recipients with aHUS who received off-label therapy with anti-C5, including 12 cases, which have not been reported yet. Nine patients, all carrying a complement genetic abnormality associated with a high risk of aHUS recurrence, received prophylactic anti-C5 therapy to prevent posttransplant recurrence. Eight of them had a successful recurrence-free posttransplant course and achieved a satisfactory graft function, while the remaining patient experienced early arterial thrombosis of the graft. Thirteen renal transplant recipients were given anti-C5 for posttransplant aHUS recurrence. A complete reversal of aHUS activity was obtained in all of them. Importantly, the delay of anti-C5 initiation after the onset of the aHUS episode inversely correlated with the degree of renal function improvement. Three patients in whom anti-C5 was subsequently stopped experienced a relapse. Altogether these data suggest that long-term eculizumab is highly effective for preventing and treating posttransplant aHUS recurrence. Our study also indicates that anti-C5 should be promptly started if a recurrence occurs.
© Copyright 2012 The American Society of Transplantation and the American Society of Transplant Surgeons.
Source: PubMed